Ophthalmic disturbances in children with sensorineural hearing loss

from the European Archives of Oto-Rhino-Laryngology

Abstract Co-incidence of sensorineural hearing loss and ophthalmic abnormalities has a tremendous influence on development of children. This study was done to determine the nature and prevalence of ophthalmic disturbances in children with congenital sensorineural hearing loss. In a descriptive cross-sectional study, complete ophthalmologic examinations such as assessment of visual acuity, cycloplegic refraction, ocular motility examination, slit lamp examination and indirect funduscopy on 50 children with sensorineural hearing loss (determined by ABR), were performed. Thirty-two percent of cases had at least one kind of ophthalmic disturbances. Refractive errors were the most common abnormalities (28%), including astigmatism (12%), myopia and astigmatism (8%), and hyperopia (8%). Three (6%) cases had ocular motility disturbance including one case of esotropia, one case of exophoria and one case of exotropia. Twenty-four percent of cases had retinal abnormalities, including suspected Rubella retinopathy (6%), pigmentary changes suspect to retinitis pigmentosa (4%), optic nerve hypoplasia (4%), and finally poor fovea reflex (10%).

Evidence of Vestibular and Balance Dysfunction in Children With Profound Sensorineural Hearing Loss Using Cochlear Implants

from Laryngoscope

Abstract:
Objectives/Hypothesis: Similarities between the peripheral auditory and vestibular systems suggest that children with sensorineural hearing loss (SNHL) may demonstrate vestibular and balance impairments. This hypothesis was studied in 40 children with severe to profound SNHL and unilateral cochlear implants.

Study Design: Prospective cross-sectional study with repeated measures.

Methods: Vestibular function was assessed with caloric, rotational, and vestibular evoked myogenic potential testing; balance was assessed using the balance subset of the Bruininks-Oseretsky Test of Motor Proficiency-II, a standardized test of static and dynamic balance.

Results: Horizontal semicircular canal function was abnormal in response to a caloric stimulus in 50% (16/32), with a large proportion of those [6/16 (38%)] reflecting mild to moderate unilateral abnormalities. In comparison, horizontal semicircular canal function in response to rotation was abnormal in 38% (14/37). Saccular function was absent bilaterally in 5/26 (19%) and unilaterally in 5/26 (19%) with vestibular evoked myogenic potential. Age standardized balance abilities were significantly poorer in the study population [[mu] = 12.9 +/- 5(SD)] compared with normal hearing controls [[mu] = 17 +/- 5(SD); P = .0006] and correlated best with horizontal canal function in response to a rotational stimulus (P = .004; R2 = 0.24). SNHL from meningitis was associated with worse balance function than other etiologies.

Conclusions: Vestibular and balance dysfunction occurred in >1/3 of children with SNHL and cochlear implants, and is highly dependent on etiology. Although compliance with all tests was high, rotational chair testing, which assesses higher frequency motion (0.25-5 Hz) and thus more “real world” vestibular function, correlated best with dynamic balance. For this reason, rotational chair testing may represent the test of choice in this population, particularly given that it is amenable to testing children of all ages.

Prognostic Value of Initial Electronystagmography Findings in Idiopathic Sudden Sensorineural Hearing Loss Without Vertigo

from Otology & Neurotology

Objective: We used electronystagmography (ENG) to characterize recurrent hearing loss and its progression to definite Meniere’s disease in patients with idiopathic sudden sensorineural hearing loss (SSHL) without subjective vertigo.

Methods: We reviewed medical records of 1,334 patients with unilateral hearing loss initially diagnosed with idiopathic SSHL between 1985 and 2003 at our university hospital. Of the 1,334 patients, we examined 127 (86 with low-tone and 41 with high-tone SSHL) who underwent ENG within 30 days of the initial diagnosis and who could be followed up during the long term.

Results: Spontaneous nystagmus (SN) was observed in approximately half of the vertigo-unaccompanied group. Long-term follow-up with a mean of 67 months revealed that the recurrence rate of hearing loss was 51.2% in low-tone SSHL patients with SN. The recurrence rate of hearing loss was 27.9% in low-tone SSHL patients without SN. Progression to Meniere’s disease occurred in 14.0% of the low-tone-type and 12.5% of the high-tone-type patients when SN was detected. In contrast, in both the low-tone-type and high-tone-type groups, none developed Meniere’s disease when SN was absent.

Conclusion: Our results suggest that the initial ENG findings could provide prognostic information for idiopathic SSHL patients, even those who have no vestibular symptoms at the first visit.

Inconsistent Management Of Sudden Sensorineural Hearing Loss Among Clinicians

from Medical News Today.com

Patients seeking care for sudden sensorineural hearing loss (SSHL) are receiving inconsistent care due to lack of established clinical practice guidelines, says new research presented at the 2008 American Academy of Otolaryngology - Head and Neck Surgery Foundation (AAO-HNSF) Annual Meeting & OTO EXPO in Chicago, IL.

Evaluation of Pediatric Sensorineural Hearing Loss With Magnetic Resonance Imaging

from Archives of Otolaryngology–Head and Neck Surgery

Objective To evaluate the incidence and type of intracranial and inner ear abnormalities in children with sensorineural hearing loss (SNHL) identified with magnetic resonance imaging (MRI) and stratified by the degree and type of SNHL.

Design Retrospective review of medical records and MRIs.

Setting Tertiary care children’s hospital.

Patients A total of 227 children aged 1 month to 17 years (mean age, 5.3 years; male to female ratio, 1:1) with a diagnosis of SNHL underwent MRI from June 1,1996, to June 1, 2002. Of these children, 170 had clinical information available and technically adequate MRIs and were included in the study.

Intervention Magnetic resonance imaging.

Main Outcome Measure Identification of an abnormality of the intracranial contents, inner ear, and cochlear nerve.

Results Of the 170 children, 101 (59%) had bilateral SNHL and 69 (41%) had unilateral SNHL, comprising 271 ears with SNHL. Abnormalities of the inner ear were found in 108 ears (40%) with 87 (32%) having abnormalities of the cochlea, which were considered mild in 63 (23%) and moderate to severe in 24 (9%). Forty-nine of 271 ears (18%) with SNHL demonstrated an either absent (26/49 [53%]) or deficient (23/49 [47%]) cochlear nerve. Ears with severe and profound SNHL had more abnormalities than ears with mild and moderate SNHL (66/138 [48%] vs 23/80 [29%]; P = .006), and children having ears with unilateral moderate, severe, or profound SNHL had more inner ear abnormalities than children with bilateral moderate, severe, or profound SNHL (28/45 [62%] vs 54/144 [38%]; P = .004).

Conclusions The overall incidence of inner ear abnormalities in ears of children with SNHL evaluated by MRI is 40%. The most common abnormalities seen were an abnormal cochlea and abnormal cochlear nerve. Children with severe and profound SNHL have a greater percentage of inner ear anomalies than children with mild or moderate SNHL. Children with unilateral hearing loss have a greater percentage of inner ear anomalies than children with bilateral SNHL.

Evidence of Vestibular and Balance Dysfunction in Children with Profound Sensorineural Hearing Loss Using Cochlear Implants

from Laryngoscope

Abstract:
Objectives/Hypothesis: Similarities between the peripheral auditory and vestibular systems suggest that children with sensorineural hearing loss (SNHL) may demonstrate vestibular and balance impairments. This hypothesis was studied in 40 children with severe to profound SNHL and unilateral cochlear implants.

Study Design: Prospective cross-sectional study with repeated measures.

Methods: Vestibular function was assessed with caloric, rotational, and vestibular evoked myogenic potential testing; balance was assessed using the balance subset of the Bruininks-Oseretsky Test of Motor Proficiency-2, a standardized test of static and dynamic balance.

Results: Horizontal semicircular canal function was abnormal in response to a caloric stimulus in 50% (16/32), with a large proportion of those [6/16 (38%)] reflecting mild to moderate unilateral abnormalities. In comparison, horizontal semicircular canal function in response to rotation was abnormal in 38% (14/37). Saccular function was absent bilaterally in 5/26 (19%) and unilaterally in 5/26 (19%) with vestibular evoked myogenic potential. Age standardized balance abilities were significantly poorer in the study population [[mu] = 12.9 +/- 5(standard deviation)] compared with normal hearing controls [[mu] = 17 +/- 5(standard deviation); P = .0006] and correlated best with horizontal canal function in response to a rotational stimulus (P = .004; R2 = 0.24). SNHL from meningitis was associated with worse balance function than other etiologies.

Conclusions: Vestibular and balance dysfunction occurred in >1/3 of children with SNHL and cochlear implants, and is highly dependent on etiology. Although compliance with all tests was high, rotational chair testing, which assesses higher frequency motion (0.25-5 Hz) and thus more “real world” vestibular function, correlated best with dynamic balance. For this reason, rotational chair testing may represent the test of choice in this population, particularly given that it is amenable to testing children of all ages.

Phenotype/Genotype Correlations in a DFNB1 Cohort With Ethnical Diversity

from Laryngoscope

Abstract:
Objectives/Hypothesis: The aim of this study was to 1) determine the prevalence of DFNB1 in a cohort of children with prelingual nonsyndromic sensorineural hearing loss (HL), 2) study phenotype/genotype correlations, and 3) establish guidelines for genetic counseling of DFNB1.

Study Design: Prospective cohort study.

Methods: A total of 119 unrelated children (107 sporadic and 12 familial cases) with prelingual nonsyndromic HL underwent mutational screening for DFNB1 in the noncoding and coding exons of GJB2, in addition to the del(GJB6-D13S1830) mutation of GJB6. Information regarding demographics, HL, developmental milestones, inner ear high resolution computed tomography, hearing habilitation, and associated phenotypic manifestations were collected in probands with biallelic pathogenic mutations.

Results: The prevalence of DFNB1 in cases of prelingual nonsyndromic HL was 26% (25% in sporadic and 50% in familial cases). In regards to ethnicity, 19 probands were white and 12 probands of Hispanic ancestry had a mixed racial origin (black, Native-American, white). Greater allelic heterogeneity was shown with Hispanics of mixed descent exhibiting 10 of 12 GJB2 allelic variants, whereas whites had 4 of 10 allelic variants (Fisher exact test, P = .033); both ethnic groups had the GJB6 deletion. The frequency of deaf carriers of the most commonly found mutation (c.35delG) was 8% and higher than that of expected for the general population (Fisher exact test, P = .015). The hearing phenotype was variable in terms of degree of impairment (from mild to profound), onset, symmetry and progression, and there was no correlation with any specific genotype class. DFNB1 probands had normal gross motor development, and the frequency of computed tomography abnormalities of the inner ear was low at 8%. No other specific associated phenotypic manifestations were identified.

Conclusions: DFNB1 is the most common identifiable etiology of nonsyndromic prelingual deafness both in sporadic and familial cases in this cohort with ethnic diversity. The greater allelic variability observed in Hispanics and the high frequency of deaf probands carrying a single allelic variant of DFNB1 support extending the screening to noncoding regions of GJB2 and to the remaining DFNB1 locus. Most probands have a congenital HL that is stable, symmetrical and without associated manifestations, but the audiometric profile should not be the only criteria for offering mutational screening of DFNB1 because of the observed variability. These data can be applied to direct the clinical evaluation and effectively counsel families of children with DFNB1.

In Search of the DFNA11 Myosin VIIA Low- and Mid-Frequency Auditory Genetic Modifier

from Otology & Neurotology

Abstract:
Objectives: To evaluate the auditory, vestibular, and retinal characteristics of a large American DFNA11 pedigree with autosomal dominant progressive sensorineural hearing loss that first impacts the low- and mid-frequency auditory range. The pedigree (referred to as the HL2 family) segregates a myosinVIIA (MYO7A) mutation in exon 17 at DNA residue G2164C (MYO7AG2164C) that seems to be influenced by a genetic modifier that either rescues or exacerbates the MYO7AG2164C alteration. DNA analysis to examine single-nucleotide polymorphisms in 2 candidate modifier genes (ATP2B2 and Wolfram syndrome 1 [WFS1]) is summarized in this report.

Study Design: Family study.

Results: The degree of low- and mid-frequency hearing loss in HL2 family members segregating the MYO7AG2164C mutation varies from mild to more severe, with approximately the same number of HL2 family members falling at each end of the severity spectrum. The extent of hearing loss in HL2 individuals can vary between family generations. Differences in the degree of hearing loss in MYO7AG2164C HL2 family members may be mirrored by vestibular function in at least 2 of these same individuals. The single-nucleotide polymorphisms examined within ATP2B2 and WFS1 did not segregate with the mild versus more severe auditory phenotype.

Conclusion: The severity of the auditory and vestibular phenotypes in MYO7AG2164C HL2 family members may run in parallel, suggesting a common modifier gene within the inner ear. The putative MYO7AG2164C genetic modifier is likely to represent a common polymorphism that is not linked tightly to the MYO7A mutation on the MYO7A2164C allele.

Risk Factors of Sensorineural Hearing Loss in Patients With Ischemic Stroke

from Otology & Neurotology

Abstract:
Objectives: We investigated risk factors of sensorineural hearing loss (SHL) in patients with early stage of ischemic stroke (IS).

Methods: Our study consisted of 60 patients with IS who were treated at Department of Neurology, Medical University of Gdansk between 2006 and 2007. A multivariate stepwise linear regression was used to identify the risk factors that were related to SHL as measured by pure-tone average (0.5, 1, and 2 kHz), the high-tone average (4, 6, and 8 kHz), the pure-tone middle-tone average (0.5, 1, 2, and 4 kHz), and the overall average (0.5, 1, 2, 4, 6, and 8 kHz). The following factors were included in the analysis: age, sex, smoking habit, addiction to alcohol, presence of tinnitus and vestibular symptoms, arterial hypertension, cardiac ischemic disease, hyperlipidemia, diabetes, number of risk factors, neurologic state at the admission, localization of stroke, cause of stroke, number of ischemic focuses, the side and diameter of ischemic focuses, the result of directional hearing acuity angle test, results of certain laboratory tests, blood morphology parameters, and coagulogram. Values for p less than 0.05 were considered significant.

Results: Our analysis suggests the presence of the following risk factors of SHL in patients with IS: 1) older age (>60 yr); 2) male sex; 3) presence of tinnitus; 4) arterial hypertension; 5) lacunar type of stroke; 6) presence of multiple ischemic focuses; 7) presence of bilateral ischemic focuses; changes in blood cell count; and 9) disturbances of agglutination.

Conclusion: The highest risk of hearing loss in the group of IS patients occurred at older people, particularly men with tinnitus, lacunar stroke, multiple, bilateral ischemic focuses, and arterial hypertension. A negative statistically significant influence of decreased level of red cells, platelets, and hematocrit value on hearing loss was found. Abnormally lowered levels of activated partial thromboplastin time, activated partial thromboplastin time ratio, prothrombin index, and abnormal lowered level of international normalized ratio statistically impaired hearing.

Idiopathic Sudden Sensorineural Hearing Loss

from the New England Journal of Medicine

A healthy 58-year-old woman answers the telephone and realizes that her hearing is diminished on the left side. She notices aural fullness and loud tinnitus in the affected ear. Later that day she has several hours of mild vertigo that clears by the following morning. Over the next few days, repeated self-administered ear cleaning with the use of an over-the-counter kit does . . .

Spectrum of GJB2 mutations causing deafness in the British Bangladeshi population

from Clinical Otolaryngology

Objective: Mutations in Gap Junction Beta 2 (GJB2) (the gene encoding the protein Connexin 26) have been found to be a major cause of non-syndromic sensorineural recessive deafness. The mutations in GJB2 causing hearing impairment vary in different populations. The aim of this study was to determine the prevalence and spectrum of GJB2 mutations in prelingual deafness in a population of Bangladeshi origin in the UK.

Design: Cross-sectional survey.

Setting: Community based audiology clinic and tertiary level genetics department.

Methods: Fifty-three families (67 patients) with sensorineural hearing loss of unknown cause were included in the study. Detailed history and examination excluded syndromic and environmental causes of hearing loss in the subjects as far as possible. Genetic analysis was performed, specifically looking for mutations in the GJB2 gene.

Results: Of the 53 families, 14 were confirmed to have biallelic pathogenic mutations in GJB2 (26%). The most common mutations of GJB2 in this population were W24X, IVS1+1, M1V, W77X and Q124X, W24X being the most common mutation seen in 57% of patients.

Conclusion: Mutations in GJB2 are responsible for over one quarter of non-syndromic sensorineural deafness in the British Bangladeshi population. It is recommended that all Bangladeshi patients with non-syndromic hearing loss should be first tested for GJB2 mutations before requesting other aetiological investigations.

Hearing rehabilitation in a patient with sudden sensorineural hearing loss in the only hearing ear

from the Journal of the American Academy of Audiology

A case report is presented of a 62-year-old software product manager who had normal hearing in one ear and a congenital profound hearing loss in the other ear and then sustained a sudden sensorineural hearing loss in the only hearing ear. The approach to amplification decisions, cochlear implant evaluation, and rehabilitation options are discussed. Providing aural rehabilitation and continually updating and providing new amplification options and accessories are described.

Histopathology of Nonsyndromic Autosomal Dominant Midfrequency Sensorineural Hearing Loss

from Otology & Neurotology

Abstract:
Background: Autosomal dominant, nonsyndromic, midfrequency sensorineural hearing loss (SNHL) is a well-known clinical entity. There are no reported histopathologic studies of temporal bones from individuals with such a hearing loss.

Objectives: To describe the otopathology in 2 affected individuals from 2 different kindreds with nonsyndromic, dominant, midfrequency SNHL.

Material and Methods: Both subjects belonged to multigenerational families with nonsyndromic, autosomal dominant SNHL showing a cookie-bite pattern. Temporal bones were removed at autopsy and studied by light microscopy. Cytocochleograms were constructed for hair cells, stria vascularis, and cochlear neuronal cells.

Results: Subject 1 (a 77-yr-old man) from Kindred A was diagnosed in early childhood with an SNHL that was progressive, reaching profound levels by adulthood. Both cochleae showed complete loss of inner and outer hair cells, moderate to severe diffuse atrophy of the stria vascularis, and severe loss of cochlear neurons, including the peripheral dendrites. The hearing loss in Subject 2 (an 82-yr-old man from Kindred B) began in late childhood, was slowly progressive, and involved the higher frequencies later in life. Histopathology showed loss of outer and inner hair cells in the basal turn of the cochlea, moderate to severe loss of stria vascularis, but relative preservation of peripheral dendrites and cochlear neurons.

Conclusion: The main histopathologic abnormalities were loss of hair cells, stria vascularis, and cochlear neurons in 1 case and loss of hair cells and stria vascularis in the second case. Our results are consistent with the hypothesis that dysfunction and loss of hair cells may have been the primary histopathologic correlate for the midfrequency hearing losses in these 2 subjects.

Inner-ear obliteration in ulcerative colitis patients with sensorineural hearing loss

from the Journal of Laryngology and Otology

Objective: Systemic autoimmune diseases, including ulcerative colitis, may involve the inner ear. Several ulcerative colitis cases presenting with sensorineural hearing loss have been reported. We report the T2-weighted, three-dimensional, inner-ear magnetic resonance imaging findings in the inner ears of two such patients.

Methods: Case reports and a review of the literature concerning autoimmune disease and sensorineural hearing loss are presented.

Results: We describe two cases of ulcerative colitis with sensorineural hearing loss in which three-dimensional magnetic resonance imaging revealed obliteration of the inner ear. Those inner ears with obliteration had severe hearing loss, and responded poorly to steroid therapy.

Conclusion: To our knowledge, there has been no previous published report of the T2-weighted, inner-ear magnetic resonance imaging findings of cases of ulcerative colitis with sensorineural hearing loss. This paper represents the first published report in the world literature of inner-ear obliteration in such patients. Three-dimensional magnetic resonance imaging is beneficial in elucidating the pathophysiology of the inner-ear involvement seen in ulcerative colitis.

Cochlear Implants Stimulate Activity-Dependent CREB Pathway in the Deaf Auditory Cortex: Implications for Molecular Plasticity Induced by Neural Prosthetic Devices

from Cerebral Cortex

Neural activity modulates the maturation of synapses and their organization into functional circuits by regulating activity-dependent signaling pathways. Phosphorylation of cyclic AMP/Ca2+-responsive element-binding protein (CREB) is widely accepted as a stimulus-inducible event driven by calcium influx into depolarized neurons. In turn, phosphorylated CREB (pCREB) activates the transcription of brain-derived neurotrophic factor (BDNF), which is needed for synaptic transmission and long-term potentiation. We examined how these molecular events are influenced by sensorineural hearing loss and long-term reactivation via cochlear implants. Sensorineural hearing loss reduced the expression of pCREB and BDNF. In contrast, deafened animals subject to long-term, unilateral intracochlear electrical stimulation exhibited an increased expression of pCREB and BDNF in the contralateral auditory cortical neurons, relative to ipsilateral ones. These changes induced by cochlear implants are further accompanied by the activation of the mitogen-activated protein kinase (MAPK) signaling pathway, which has been implicated in long-lasting forms of synaptic plasticity. Because CREB and BDNF are critical modulators of synaptic plasticity, our data describe for the first time possible molecular candidate genes, which are altered in the auditory cortex, following cochlear implantation. These findings provide insights into adaptive, molecular mechanisms recruited by the brain upon functional electrical stimulation by neural prosthetic devices.

Course of hearing recovery according to frequency in patients with acute acoustic sensorineural hearing loss

from the International Tinnitus Journal

Through pure-tone audiometry, we studied the course of hearing recovery in 24 ears of 20 men (ages 18-48 years) who had acute acoustic sensorineural hearing loss (ASHL). All subjects were members of the Japanese Self-Defense Force. The hearing level in 5 ears returned to normal, the hearing level of 13 ears recovered but was not within the normal range, and the hearing level of 6 ears was unchanged. The time from noise exposure to presentation was longer in patients with unchanged hearing than in other patients. Recovery of hearing was poorest at 4,000 Hz, followed by 8,000 and 2,000 Hz. We concluded that hearing in patients with acute ASHL is likely to return to normal when the hearing level at 4,000 Hz recovers gradually; partial recovery of hearing is expected when the hearing level at 4,000 Hz reaches an early plateau.

Cochlear microphonics in sensorineural hearing loss: Lesson from newborn hearing screening

from the International Journal of Pediatric Otorhinolaryngology

the diagnostic dilemma surrounding the presence of cochlear microphonics (cm) coupled with significantly elevated auditory brainstem response (abr) thresholds in babies failing the newborn hearing screening is highlighted. a case report is presented where initial electo-diagnostic assessment could not help in differentiating between auditory neuropathy/auditory dys-synchrony (an/ad) and sensorineural hearing loss (snhl). in line with the protocol and guidelines provided by the national newborn hearing screening programme in the uk (nhsp) an/ad was suspected in a baby due to the presence of cm at 85 dbnhl along with click evoked abr thresholds of 95 dbnhl in one ear and 100 dbnhl in the other ear. significantly elevated thresholds for 0.5 and 1 khz tone pip abr fulfilled the audiological diagnostic criteria for an/ad. however, the possibility of a snhl could not be ruled out as the 85 dbnhl stimuli presented through inserts for the cm would have been significantly enhanced in the ear canals of the young baby to exceed the threshold level of the abr that was carried out using headphones. snhl was eventually diagnosed through clinical and family history, physical examination and imaging that showed enlarged vestibular aqueducts. presence of cm in the presence of very high click abr thresholds only suggests a pattern of test results and in such cases measuring thresholds for 0.5 and 1 khz tone pip abr may not be adequate to differentiate between snhl and other conditions associated with an/ad. there is a need for reviewing the existing an/ad protocol from nhsp in the uk and new research to establish parameters for cm to assist in the differential diagnosis. a holistic audiological and medical approach is essential to manage babies who fail the newborn hearing screening.

Auditory steady-state response and auditory brainstem response thresholds in children

from the European Archives of Oto-Rhino-Laryngology

Abstract The inclusion of the auditory steady-state response (ASSR) into test-batteries for objective audiometry has allowed for clinical comparisons with the most widely used procedure, the auditory brainstem response (ABR). The current study describes ASSR and ABR thresholds for a group of infants and young children with various types and degrees of hearing loss. A sample of 48 subjects (23 female) with a mean age of 2.8 ± 1.9 years SD were assessed with a comprehensive test-battery and classified according to type and degree of hearing loss. Thresholds were determined with a broadband click-evoked ABR and single frequency ASSR evoked with continuous tones (0.25–4 kHz) amplitude modulated (67–95 Hz). Mean difference scores (±SD) between the ABR and high frequency ASSR thresholds were 9.8 (±11), 3.6 (±12) and 10.5 (±12) dB at 1, 2 and 4 kHz, respectively. An ASSR mean threshold for 2–4 and 1–4 kHz compared to the ABR threshold revealed an average difference of 7 (±9) and 7.9 (±8) dB, respectively. The overall correlation between the ABR and ASSR thresholds was highest for the mean ASSR thresholds of 2–4 and 1–4 kHz (r = 0.92 for both conditions). Correlations between the ABR and individual ASSR frequencies were slightly less (0.82–0.86). The average of the 2–4 kHz ASSR thresholds correlated best with the click-evoked ABR for all categories of hearing loss except for the sensorineural hearing loss category for which the 1–4 kHz ASSR average was better correlated to ABR thresholds. Findings demonstrate the reliability of verifying high frequency ASSR thresholds with a click-evoked ABR as an important cross-check in infants for whom behavioural audiometry may not be possible.

Czech dysplasia: Report of a large family and further delineation of the phenotype

from the American Journal of Medical Genetics Part A

Abstract
Czech dysplasia (OMIM 609162) is a recently delineated COL2A1 disorder characterized by early-onset progressive pseudorheumatoid arthritis, platyspondyly, short third and fourth metatarsals, normal height, and the absence of ophthalmological problems or cleft palate. Czech dysplasia is caused by a specific missense mutation (R275C, c.823C > T) in the triple helical domain of the COL2A1 gene. We report on a large family with 11 patients with typical Czech dysplasia and sensorineural hearing loss. Hearing loss has hitherto not been considered as a major manifestation of Czech dysplasia. Mutation analysis documented the COL2A1 c.823C > T (R275C) mutation in all affected individuals. Thus, Czech dysplasia is possibly caused exclusively by the R275C mutation, which is a unique situation among the COL2A1 disorders. The family provides further evidence for the remarkably uniform manifestation of the clinical and radiological abnormalities and adds hearing loss to the list of major anomalies of Czech dysplasia. © 2008 Wiley-Liss, Inc.

High-Frequency Sensorineural Hearing Loss After Stapedectomy

from Otology & Neurotology

Objective: To describe the pattern and duration of high frequency sensorineural hearing loss after stapedectomy.

Study Design: Retrospective case series.

Setting: Tertiary referral center.

Patients: All patients who underwent stapedectomy by the senior author during the period between January 1, 1998, and October 1, 2005, with preoperative, 4- to 6-week postoperative, and at least 9-month postoperative audiograms were included. Fifty-three patients met the inclusion criteria, with surgeries performed on 61 ears.

Intervention(s): Stapedectomy was performed using a CO2 laser.

Main Outcome Measure(s): Mean preoperative and postoperative pure-tone bone thresholds, mean preoperative and postoperative pure-tone air thresholds, and hearing outcomes for 4,000 Hz bone conduction (BC) and 8,000 Hz air conduction (AC) based on the patient’s age and interval after the operative procedure.

Results: Mean BC thresholds at 4,000 Hz BC worsened by 6 dB at 4 to 6 weeks and improved by 3 dB by 9 months. There was an 8-dB average loss at 8,000 Hz AC at 4 to 6 weeks with a gain of 4 dB by 9 months. Patients older than 40 years were 4 times more likely to experience early loss at 4,000 Hz BC when preoperative thresholds were held constant. The late outcome for hearing loss was dependent more on the preoperative threshold than was the age of the patient. At 8,000 Hz AC, the preoperative hearing threshold was a predictor of early and late hearing loss at 8,000 Hz.

Conclusion: High-frequency sensorineural hearing loss is initially seen after stapes surgery, and improvement does occur over time. When preoperative hearing threshold is held constant, patients older than 40 years are more likely to experience short-term hearing loss at high frequencies than patients younger than 40 years. Age was not a predictor of postoperative high-frequency hearing loss in the long term.

Sensorineural Hearing Loss After Radiotherapy for Head and Neck Tumors: A Prospective Study of the Effect of Radiation

from Otology & Neurotology

Introduction: In the treatment of head and neck cancers, the cochlea may be damaged if it is within the radiotherapy (RT) area; however, the severity and mechanism of such damage have yet to be clearly defined. The purpose of this study was to analyze the rates of early- and late-stage sensorineural hearing loss (SNHL) in patients receiving RT due to head and neck cancer and to investigate the reliability of distortion product otoacoustic emissions (DPOAEs) measurements and audiometric evaluation.

Study Design: Prospective.

Method: The study included 38 ears of 19 patients scheduled for RT due to head and neck cancer diagnoses. The patients received RT at a fractioned dose of 200 cGy (5 d/wk) and were evaluated pretreatment and posttreatment (1st and 12th mo), both audiometrically and with DPOAE measurement. Any decrease greater than 10 dB was considered SNHL. The amplitudes of DPOAE measurements were statistically compared.

Results: The audiometric evaluation performed in the 1st posttreatment month showed no SNHL in any of the patients, whereas in the 12th month, 47% of the ears had SNHL. In all the patients that developed SNHL, the amplitudes obtained in DPOAE measurements in the first posttreatment month were statistically significantly lower.

Conclusion: The results of the present study show that DPOAE measurement is a reliable method for determining which patients are at risk of developing SNHL in the early post-RT period.

Diagnostic tests for immunomediated hearing loss: a systematic review

from the Journal of Laryngology and Otology

Objective: To quantitatively evaluate the diagnostic accuracy of diagnostic tests for immunomediated hearing loss.

Data sources: We searched Medline and the Cochrane Database of Systematic Reviews for potentially relevant studies.

Study selection: Twenty-five studies met the inclusion criteria of this systematic review. The diagnosis of immunomediated hearing loss was based on the clinical presentation and the response to corticosteroid administration.

Data extraction: The following data were extracted from the selected studies and entered into a standardised database: population demographics; exclusion and inclusion criteria; diagnostic tests; sensitivity; specificity; the number of true positive, true negative, false positive and false negative values; therapy used, including dose and duration; and delay between symptom onset and therapy commencement.

Data synthesis: This systematic review combined data from 679 patients with immunomediated hearing loss, reported by 22 research teams. Substantial heterogeneity was found among the included studies; for this reason, summary sensitivity and specificity values were not computed.

Conclusions: The results of diagnostic tests for immunomediated hearing loss depend on many factors, and there is a risk of potential bias. This is the first time that such a systematic review has been presented; such a review is a more rigorous method of demonstrating the utility of the available diagnostic tests.

(Accepted June 04 2007)

(Online publication October 02 2007)

A systematic review of the incidence of sensorineural hearing loss in neonates exposed to Herpes simplex virus (HSV)

from the International Journal of Pediatric Otorhinolaryngology

Summary
Objective
To develop evidence-based guidelines for appropriate audiological monitoring of children born following exposure to or infection with Herpes simplex virus (HSV) for development of immediate or delayed-onset of sensorineural hearing loss (SNHL).

Data sources
A Medline search of the 1966–July 2007 database was supplemented by search of the additional database Embase (1980–July 2007). Manual search was conducted of references of identified papers and book chapters.

Study selection
Articles were sought that were longitudinal in design, to include an inception cohort of children infected with (or exposed to) HSV who were entered at a similar point at birth and followed over time with serial audiometry to identify hearing loss if it developed.

Data extraction
Patient information and audiometric data extraction from relevant articles was performed independently by all three researchers. Discrepancies were resolved by mutual consensus.

Data synthesis
Data was analyzed using descriptive statistics.

Results
Three papers reported five children with SNHL following apparent disseminated HSV-2 infections in which other obvious clinical sequelae of HSV infection and co-morbid conditions were present. Audiometric information is lacking regarding onset and progression. There are no reports of delayed-onset SNHL following perinatal or asymptomatic HSV infection.

Conclusions
The development of SNHL in children with exposure to HSV occurs rarely. Routine serological screening for HSV infection in otherwise healthy neonates newly diagnosed with SNHL is unjustified. There is insufficient data to define the incidence and natural history of SNHL in children with HSV infections. Carefully designed and conducted studies are needed to address this issue.

Receptive (aural) vocabulary development in children with permanent bilateral sensorineural hearing impairment

from the Journal of Laryngology and Otology

Objective: The receptive (aural) vocabulary development of children with binaural-aided residual hearing was investigated in a prospective longitudinal analysis (repeated measures).

Patient and methods: Thirty-three children with sensorineural hearing loss, without major co-morbidities (mean age at the first testing point, 56.2 months, standard deviation 19.0 months) were recruited from the 1994 German Goettinger Hearing Language Register. Their receptive vocabulary was measured using standardised tests (with reference to normal hearing child performance) on three separate test points (mean follow up, 18.7 months, standard deviation 5.0 months).

Results: On average, the study group scored below the normal range at the first and second tests and made a significant improvement at the last test. The degree of hearing loss was found to be significantly correlated to vocabulary performance at all time points, with increasing correlation coefficients. At the third test, significant predictive variables for successful receptive vocabulary outcome were found to be: degree of hearing loss (t = 5.43; p < 0.0001); age at diagnosis (t = 2.29; p = 0.03); and nonverbal intelligence (t = 2.82; p = 0.009).

Conclusion: If permanent childhood hearing impairment is mild and/or is detected early, and if the child grows up in a monolingual environment, the development of receptive vocabulary within the normal range is possible.

Spectral weighting strategies for hearing-impaired listeners measured using a correlational method

from the Journal of the Acoustical Society of America

Spectral weighting strategies using a correlational method [R. A. Lutfi, J. Acoust. Soc. Am. 97, 1333–1334 (1995); V. M. Richards and S. Zhu, J. Acoust. Soc. Am. 95, 423–424 (1994)] were measured in ten listeners with sensorineural-hearing loss on a sentence recognition task. Sentences and a spectrally matched noise were filtered into five separate adjacent spectral bands and presented to listeners at various signal-to-noise ratios (SNRs). Five point-biserial correlations were computed between the listeners’ response (correct or incorrect) on the task and the SNR in each band. The stronger the correlation between performance and SNR, the greater that given band was weighted by the listener. Listeners were tested with and without hearing aids on. All listeners were experienced hearing aid users. Results indicated that the highest spectral band (~2800–11 000 Hz) received the greatest weight in both listening conditions. However, the weight on the highest spectral band was less when listeners performed the task with their hearing aids on in comparison to when listening without hearing aids. No direct relationship was observed between the listeners’ weights and the sensation level within a given band. ©2008 Acoustical Society of America

A comparative study of the audiological outcomes with Retro-X (semi-implantable hearing aid system) and conventional open fitting hearing aids

from the European Archives of Oto-Rhino-Laryngology

Abstract The objective of this study was to compare the results of hearing augmentation with the Retro-X semi-implantable hearing aid to a conventional non-implantable open canal hearing aid using the same software technology (Titan-X, auric Hearing Systems, HiKaNo.:13.20.03.0047). Nineteen subjects (20 ears) with mild to moderate high-frequency sensory-neural hearing loss were included in this clinical study. All the subjects were first fitted with a conventional open canal hearing aid (Titan-X). After a period of 4–6 weeks audiological evaluations were performed using standardized speech tests in quiet and noise. Subjective evaluation was performed with the help of standardized questionnaires. After this phase the patients received the semi-implantable Retro-X device under local anaesthesia and 4 weeks later were fitted with a hearing module with the same software technology as the conventional Titan-X hearing aid. Four weeks following the first fitting the same audiological evaluations were performed under similar conditions and the patients were evaluated again using the same questionnaires in order to compare the results of the Retro-X semi-implantable system with the conventional open canal hearing aid. Audiological evaluations revealed better results with the semi-implantable Retro-X in the adaptive speech in noise test; however, the results of the monosyllabic word test in quiet were similar for the Retro-X and conventional open canal hearing aid. The patients subjectively preferred the improved wearing comfort of the semi-implantable device. The Retro-X semi-implantable hearing aid provides better speech perception in noise and higher wearing comfort in comparison to the conventional open canal hearing aids. Considering the simple implantation procedure under local anaesthesia with low complication rate, Retro-X is an alternative to the conventional open canal hearing aids in patients with mild to moderate high frequency sensory-neural hearing loss.

Otological findings among Nigerian children with sickle cell anaemia

from the International Journal of Pediatric Otorhinolaryngology

Background/Aim
Various degrees of hearing loss have been associated with sickle cell anaemia, especially of the sensorineural type (SNHL). However, there is little information on hearing pattern among sickle cell children in Nigeria. This study is to determine the prevalence of sensorineural hearing loss (SNHL) among children with sickle cell anaemia (SCA).

Patients and methods
Eighty (80) stable children aged 4–15 with Hbss attending the pediatric sickle cell clinic and also 60 control patients with HbAA, matched for age, sex at the pediatric general medical clinic of the University of Ilorin teaching hospital, Ilorin, Nigeria, all had prospective study of their pure tone audiological assessment (PTA) and tympanometric evaluations done over a year period.

Results
Their age range was 4–15 years with a mean of 9.4 for the Hbss and 9.7 for the control group. The male/female ratio was 1.3:1 and 1.5:1 for SCA and control subjects respectively.

25 subjects (50 ears) had abnormal audiograms among the SCA subjects and OME was the cause in 22 subjects and only three (3) had mild SNHL which was bilateral. However, in the control group 15 had abnormal audiograms and all were due to OME and none had SNHL. OME was bilateral in 19 subjects with SCA, two on the left and only one on the right. In the control group, 11 of the OME was bilateral and only four were on the left side. The prevalence of SNHL was 3.8% and OME was 27.5%.

Conclusion
We have found a prevalence rate for SNHL of 3.8% for 80 subjects with HbSS, and all cases have been a mild bilateral high frequency SNHL. Our findings suggested that SNHL is uncommon in early childhood, specifically during the years of language acquisition and early schooling. This could mean an age dependant prevalence rate of SNHL among SCA patients. However, no difference in the incidence of OME among both groups which can lead to educational difficulties from the resultant speech and language defects.

Maternal Thyroid Autoantibodies during the Third Trimester and Hearing Deficits in Children: An Epidemiologic Assessment

from AJNR - American Journal of Epidemiology

Elevated maternal thyroid autoantibodies during pregnancy are linked to infertility, miscarriage, and neurodevelopmental deficits such as in cognitive function. It has not been established whether autoantibodies to thyroid peroxidase are associated with sensorineural hearing loss (SNHL). The authors tested stored third-trimester maternal serum specimens of 1,736 children for thyroid peroxidase autoantibodies (TPOaAb) by using an enzyme-linked immunosorbent assay technique. The children participated at the Baltimore, Maryland, site of the Collaborative Perinatal Project, which enrolled pregnant women in 1959–1965. An audiology examination was administered to the children at 8 years of age and was used to identify cases of SNHL. Compared with 4.3% of the other children, 22.7% of the children whose mothers had elevated TPOaAb (62.5 IU/ml) had SNHL. The difference was significant after controlling for maternal race, age, and hypothyroidism (exact prevalence odds ratio = 7.5, 95% confidence interval: 2.4, 23.3). When a lower cutoff of TPOaAb 31.25 IU/ml was used, there continued to be an association with SNHL (exact prevalence odds ratio = 5.7, 95% confidence interval: 2.1, 15.6). The direction and magnitude of the association were similar when an alternative case definition of SNHL was used. These data suggest that antenatal exposure to maternal TPOaAb during the third trimester of pregnancy is associated with impaired auditory development.

Auditory cortical activation and speech perception in cochlear implant users

from the Journal of Laryngology and Otology

Cochlear implantation is generally accepted as a successful means of restoring auditory sensation to profoundly deaf individuals. Although most patients can expect a satisfactory outcome following implantation, some have poor speech perception outcomes. This investigation used [18F]-fluorodeoxyglucose positron emission tomography to measure cortical activity resulting from auditory stimulation in seven ‘good’ and four ‘poor’ cochlear implant recipients. Activations were significantly greater in both the primary and association cortices in the good compared with the poor implant users. We suggest that the ability to access the more specialised speech processing abilities of the auditory association cortices helps determine outcome following cochlear implantation.

Bilateral, mixed hearing loss with a predominant sensorineural component in Larsen’s syndrome

from the Journal of Laryngology and Otology

Background: Larsen’s syndrome is primarily known as an orthopaedic disease. However, it has been found to be associated with conductive hearing loss caused by ossicular malformation. The possibility of a sensorineural hearing impairment as an additional part of this syndrome has been rather neglected in the past.

Method: Case report and literature review.

Patient and results: We present a teenage boy suffering from typical Larsen’s syndrome. Despite no history of recurrent middle-ear infections, he showed a bilaterally symmetrical, mixed hearing loss dominated by an extensive sensorineural component. In order to review the corresponding literature, a Medline search was performed using the criteria ‘Larsen’s syndrome’ and ‘hearing loss/deficit’.

Conclusion: The literature review showed the presented case to be the first sufficiently documented report describing a predominant sensorineural hearing loss in a Larsen’s syndrome patient. Although a specific association of Larsen’s syndrome with sensorineural hearing loss is still speculative, this case might give grounds for further examinations of this rare finding.