Form Of Sensorineural Hearing Loss Research Articles

Состояние слуховой функции у детей-воспитанников детских домов, эвакуированных из зоны военных действий

In this study, the structure and frequency of hearing impairment in children who lived at various times in the territories of military operations were studied. The high prevalence of otorhinolaryngological diseases, combined with low detectability, entails formidable complications, leads to a decrease in the quality of life of the child and the family as a whole, and negatively affects the general level of public health. In total, during the study, 398 children aged 5 to 16 years old were examined, who were in the temporary accommodation center of the Romashka children’s resting camp. The children are students of orphanages in the Donetsk People’s Republic (DPR) and the Luhansk People’s Republic (LPR) evacuated from the war zone in the first days of the Special Operation. There were 216 girls (54.3%) and 182 boys (45.7%) among them. The average age of children was 8.5 years. Hearing loss was detected in 33 children (8.3%). Two children (0.5%) had a severe form of sensorineural hearing loss of 3–4 degrees. Two children (0.5%) had a mild form of sensorineural hearing loss of 1–2 degrees. Four children (1%) had unilateral chronic sensorineural hearing loss. The diagnosis of otitis media with effusion was established in 20 children (5%), tubo-otitis, in three patients (0.8%). Suspicion of an anomaly in the development of the middle ear, in 2 children (0.5%). Examination of children in orphanages evacuated from the DPR and LPR did not reveal a high prevalence of hearing impairment. Sensoneural hearing loss: 2%, conductive hearing loss: 6.3%. The results of the work demonstrate a wide potential for improving hearing health in the examined children. Active diagnostics of hearing pathology is shown to all children in orphanages. After treatment of conductive hearing pathology, mandatory audiological monitoring is indicated, despite the subjective improvement in hearing.

Autosomal dominant non-syndromic hearing loss maps to DFNA33 (13q34) and co-segregates with splice and frameshift variants in ATP11A, a phospholipid flippase gene

Sequencing exomes/genomes have been successful for identifying recessive genes; however, discovery of dominant genes including deafness genes (DFNA) remains challenging. We report a new DFNA gene, ATP11A, in a Newfoundland family with a variable form of bilateral sensorineural hearing loss (SNHL). Genome-wide SNP genotyping linked SNHL to DFNA33 (LOD = 4.77), a locus on 13q34 previously mapped in a German family with variable SNHL. Whole-genome sequencing identified 51 unremarkable positional variants on 13q34. Continuous clinical ascertainment identified several key recombination events and reduced the disease interval to 769 kb, excluding all but one variant. ATP11A (NC_000013.11: chr13:113534963G>A) is a novel variant predicted to be a cryptic donor splice site. RNA studies verified in silico predictions, revealing the retention of 153 bp of intron in the 3′ UTR of several ATP11A isoforms. Two unresolved families from Israel were subsequently identified with a similar, variable form of SNHL and a novel duplication (NM_032189.3:c.3322_3327+2dupGTCCAGGT) in exon 28 of ATP11A extended exon 28 by 8 bp, leading to a frameshift and premature stop codon (p.Asn1110Valfs43Ter). ATP11A is a type of P4-ATPase that transports (flip) phospholipids from the outer to inner leaflet of cell membranes to maintain asymmetry. Haploinsufficiency of ATP11A, the phospholipid flippase that specially transports phosphatidylserine (PS) and phosphatidylethanolamine (PE), could leave cells with PS/PE at the extracellular side vulnerable to phagocytic degradation. Given that surface PS can be pharmaceutically targeted, hearing loss due to ATP11A could potentially be treated. It is also likely that ATP11A is the gene underlying DFNA33.

Formins in Human Disease.

Almost 25 years have passed since a mutation of a formin gene, DIAPH1, was identified as being responsible for a human inherited disorder: a form of sensorineural hearing loss. Since then, our knowledge of the links between formins and disease has deepened considerably. Mutations of DIAPH1 and six other formin genes (DAAM2, DIAPH2, DIAPH3, FMN2, INF2 and FHOD3) have been identified as the genetic cause of a variety of inherited human disorders, including intellectual disability, renal disease, peripheral neuropathy, thrombocytopenia, primary ovarian insufficiency, hearing loss and cardiomyopathy. In addition, alterations in formin genes have been associated with a variety of pathological conditions, including developmental defects affecting the heart, nervous system and kidney, aging-related diseases, and cancer. This review summarizes the most recent discoveries about the involvement of formin alterations in monogenic disorders and other human pathological conditions, especially cancer, with which they have been associated. In vitro results and experiments in modified animal models are discussed. Finally, we outline the directions for future research in this field.

Update on Auditory Neuropathy/Dyssynchrony in Children

Auditory neuropathy/dyssynchrony (AN/AD) is a form of sensorineural hearing loss characterized by the presence of cochlear outer hair cell (OHC) function and absent or abnormal auditory neural function. This article is meant to update clinicians on best practices for diagnosis and management of AN/AD. Exciting advances in genetics present opportunities for additional evidence to classify AN/AD based on site of lesion, and may lead to additional understanding of the pathophysiology as well as prognosis. Cochlear implantation continues to be a highly effective intervention for managing AN/AD in pediatric patients. AN/AD can be a challenging condition to manage given the heterogeneity of its presentation and variety of options for management. Ultimately, clinicians must tailor treatment to the individual child which requires frequent follow-up and communication with families, educators, and other providers. Further research is needed to fully understand this disorder and advance evidence-based care for these children.

Management of autoimmune ear disease and idiopathic polyarthritis with adalimumab

Autoimmune inner ear disease (AIED) is a rare form of sensorineural hearing loss of autoimmune origin and responding to immunosuppressive therapy with corticosteroids or other immunosuppressants. Novel treatment approaches include intratympanic steroids and biological response modulators against tumor necrosis factor-α. We present a case of AIED, who concomitantly developed seronegative inflammatory polyarthritis (IPA). The treatment for IPA was initiated with methotrexate, hydroxychloroquine, and intra-articular methyl prednisolone. When there was no adequate response, the patient was started on adalimumab, which in addition to IPA also showed beneficial effects on AIED.

OCCUPATIONAL PATHOLOGY AT THE ENTERPRISES OF THE NENETS AUTONOMOUS DISTRICT: RISK FACTORS, STRUCTURE, PREVALENCE

Background. Human labor in the Arctic area is carried out in extreme climatic conditions creating an increased risk of developing health problems, including occupational diseases (OD). The purpose of the study was to investigate the working conditions, the structure and prevalence of OD in the population of the Nenets Autonomous District (NAD), the basis of the which economy is oil and gas production. Material and methods. The data of social and hygienic monitoring in the section “Working conditions and occupational morbidity” of the population of the NAD in 2008-2017 was studied. Results. The most significant harmful production factors at NAD enterprises have been established to be non-ionizing electromagnetic fields and radiations, unfavorable microclimate of workplaces, noise, intensity, and the hardness of the labor process. More than half of all district employees are involved in enterprises with satisfactory working conditions. In the NAD working population, occupational pathology is diagnosed mainly in the flight personnel of civil aviation (88% of cases) in the form of sensorineural hearing loss (96% of cases). The risk of OD development among air transport workers was higher than that of the entire population of the district (RR = 4.42; CI 2.95-6.62) and those employed in oil and gas producing enterprises (RR = 23.2; CI 8.32 - 64.4). Additional occupational health risks associated with the extraction of hydrocarbons in Arctic have not been established. Conclusion. The priority direction in the prevention of occupational pathology (mainly sensorineural hearing loss) should be the reduction of the level of industrial noise among air transport workers.

Polymorphisms of heat shock protein 70 genes (HSPA1A, HSPA1B and HSPA1L) and susceptibility of noise-induced hearing loss in a Chinese population: A case-control study.

Noise-induced hearing loss (NIHL) is the second-most frequent form of sensorineural hearing loss. When exposed to the same noise, some workers develop NIHL while others do not, suggesting that NIHL may be associated with genetic factors. To explore the relationship between single nucleotide polymorphisms (SNPs) in heat shock protein 70 (HSP70) genes (HSPA1A, HSPA1B and HSPA1L) and susceptibility to NIHL in Han Chinese workers exposed to noise, a case-control association study was carried out with 286 hearing loss cases and 286 matched with gender, age, type of work, and exposure time, drawn from a population of 3790 noise-exposed workers. Four SNPs were selected and genotyped. Subsequently, the effects of the alleles and genotypes of the three HSP70 genes (HSPA1A, HSPA1B and HSPA1L) on NIHL were analyzed by using a conditional logistic regression. A generalized multiple dimensionality reduction (GMDR) was applied to further detect an interaction between the four SNPs. Compared with the combined genotypes CC/TC, carriers of the TT genotype of rs2763979 appeared to show greater susceptibility to NIHL (P = 0.042, adjusted OR = 1.731, 95% CI 1.021–2.935). A significant interaction between rs2763979 and CNE was found (P = 0.029), and a significant association was found between TT of s2763979 and NIHL (P = 0.024, adjusted OR = 5.694, 95%CI 1.256-25.817) in the 96 dB (A)≤CNE<101 dB (A) group. The results suggest that the rs2763979 locus of the HSP70 genes may be associated with susceptibility to NIHL in Chinese individuals, and other HSP70 genes may also be susceptibility genes for NIHL, but the results must be further replicated in additional independent sample sets.

Perception of Hearing Aid-Processed Speech in Individuals with Late-Onset Auditory Neuropathy Spectrum Disorder.

Auditory neuropathy spectrum disorder (ANSD) is a form of sensorineural hearing loss, causing severe deficits in speech perception. The perceptual problems of individuals with ANSD were attributed to their temporal processing impairment rather than to reduced audibility. This rendered their rehabilitation difficult using hearing aids. Although hearing aids can restore audibility, compression circuits in a hearing aid might distort the temporal modulations of speech, causing poor aided performance. Therefore, hearing aid settings that preserve the temporal modulations of speech might be an effective way to improve speech perception in ANSD. The purpose of the study was to investigate the perception of hearing aid-processed speech in individuals with late-onset ANSD. A repeated measures design was used to study the effect of various compression time settings on speech perception and perceived quality. Seventeen individuals with late-onset ANSD within the age range of 20-35 yr participated in the study. The word recognition scores (WRSs) and quality judgment of phonemically balanced words, processed using four different compression settings of a hearing aid (slow, medium, fast, and linear), were evaluated. The modulation spectra of hearing aid-processed stimuli were estimated to probe the effect of amplification on the temporal envelope of speech. Repeated measures analysis of variance and post hoc Bonferroni's pairwise comparisons were used to analyze the word recognition performance and quality judgment. The comparison between unprocessed and all four hearing aid-processed stimuli showed significantly higher perception using the former stimuli. Even though perception of words processed using slow compression time settings of the hearing aids were significantly higher than the fast one, their difference was only 4%. In addition, there were no significant differences in perception between any other hearing aid-processed stimuli. Analysis of the temporal envelope of hearing aid-processed stimuli revealed minimal changes in the temporal envelope across the four hearing aid settings. In terms of quality, the highest number of individuals preferred stimuli processed using slow compression time settings. Individuals who preferred medium ones followed this. However, none of the individuals preferred fast compression time settings. Analysis of quality judgment showed that slow, medium, and linear settings presented significantly higher preference scores than the fast compression setting. Individuals with ANSD showed no marked difference in perception of speech that was processed using the four different hearing aid settings. However, significantly higher preference, in terms of quality, was found for stimuli processed using slow, medium, and linear settings over the fast one. Therefore, whenever hearing aids are recommended for ANSD, those having slow compression time settings or linear amplification may be chosen over the fast (syllabic compression) one. In addition, WRSs obtained using hearing aid-processed stimuli were remarkably poorer than unprocessed stimuli. This shows that processing of speech through hearing aids might have caused a large reduction of performance in individuals with ANSD. However, further evaluation is needed using individually programmed hearing aids rather than hearing aid-processed stimuli.

Current understanding of the pathogenesis of autoimmune inner ear disease: a review.

Autoimmune inner ear disease (AIED) is a poorly understood form of sensorineural hearing loss that causes bilateral, asymmetric, progressive hearing loss, sometimes with vestibular symptoms, often associated with a systemic autoimmune disease, which is noteworthy as the only sensorineural loss responsive to medical therapy. Despite much research interest of the past 25years, its aetiopathogenesis is still unproven. To succinctly consolidate research andopinion regarding the pathogenesis of autoimmune inner ear disease, in ongoing efforts to elucidate the molecular and intracellular pathways that lead to inner ear damage, which may identify new targets for pharmacotherapy. Systematic review PubMed/MEDLINE search using key terms to identify articles published between January 1980 and Apr 2014. Additionally, any landmark works discussed in this body of literature were obtained and relevant information extracted as necessary. Inclusion criterion was any information from animal or human studies with information relevant to possible aetiopathogenesis of AIED. Studies that focused on diagnosis, ameliorating symptoms or treatment, without specific information relevant to mechanisms of immune-mediated injury were excluded from this work. Articles meeting the inclusion criteria were digested and summarised. A proposed pathogenic mechanism of AIED involves inflammation and immune-mediated attack of specific inner ear structures, leading to an excessive Th1 immune response with vascular changes and tissue damage in the cochlea. Studies have identified self-reactive T cells and immunoglobulins, and have variously implicated immune-complex deposition, microthrombosis and electrochemical disturbances causing impaired neurosignalling in the pathogenesis of AIED. Research has also demonstrated abnormalities in the cytokine milieu in subjects with AIED, which may prove a target for therapy in the future. Ongoing research is needed to further elucidate the aetiopathogenesis of AIED and discern between various mechanisms of tissue injury. Large-cohort clinical studies employing IL-1 receptor blockade are warranted to determine its potential for future therapy.

Dilemmas in the management of congenital cytomegalovirus (CMV)

<h3>Background</h3> Routine antenatal ultrasound screening revealed liver calcifications, and subsequent maternal serology (IgM and IgG) was shown to be positive for cytomegalovirus (CMV). Shortly after birth he was found to have hepatosplenomegaly, petechiae and visible jaundice. Bloods within the first few hours revealed marked conjugated jaundice, thrombocytopaenia and deranged coagulation. The baby was admitted to our neonatal unit for observation and treatment and congenital CMV infection was confirmed within 24 hours by positive CMV PCR from his urine. Further investigations revealed no intracranial abnormalities, no evidence of retinitis and normal newborn hearing. Abdominal ultrasound confirmed hepatic calcification and organomegaly. <h3>Management</h3> Following discussion between neonatal and paediatric infectious disease consultants, a decision was taken to treat the infant with IV ganciclovir for 2 weeks (necessitating insertion of a percutaneous long line), followed by oral valganciclovir for 4 weeks. Weekly Viral loads were checked, together with frequent monitoring for neutropaenia. <h3>Issues</h3> This case highlighted several dilemmas in the management of congenital CMV. CMV is the most frequent congenital infection and has a UK incidence of around 3-4/1000 live births. Around 10-15% of these infants will be symptomatic, of whom up to 90% will develop long term sequelae usually in the form of sensorineural hearing loss, visual impairment or developmental delay. Some 13% of asymptomatic infants also develop long term problems. In spite of such morbidity, decisions of whom to treat, and for how long, remain controversial. Whilst there is some support for treating infants with CNS symptoms, there is a paucity of high level evidence for treating infants with other organ involvement. In addition, the adverse effects of treatment - namely requirement of central venous access with its associated complications, and potential neutropaenia – are not inconsiderable. The controversies of treatment, and uncertainty regarding long term follow-up of symptomatic and asymptomatic babies, mean such cases provide difficult management problems.

Chudley McCullough syndrome

Chudley McCullough syndrome is characterized by partial agenesis of the corpus callosum, interhemispheric cyst, cerebral and cerebellar cortical dysplasias, and hydrocephalus. This syndromic form of sensorineural hearing loss is rare. Our literature search has located 13 siblings in 6 families with this syndrome. We report a case of Chudley McCullough syndrome and discuss the relevant literature. It is amply clear from the review of literature that treatment of ventricular dilatation or drainage of arachnoid cysts in these cases will not improve the sensorineural hearing loss. A 14-month-old female child presented with bilateral profound sensorineural hearing loss. Neuroimaging revealed partial agenesis of the corpus callosum, colpocephaly, and an interhemispheric arachnoid cyst. These associations suggested a diagnosis of Chudley McCullough syndrome.

騒音性難聴２症例の側頭骨病理所見

To examine the relationship between hearing and changes in the inner ear, we investigated human temporal bone specimens from 2 patients with noise-induced hearing loss and prepared audio-cytocochleograms as described by Schuknecht et al. Patient 1 was a 50-year-old male who died of thyroid cancer and had worked at a printing house for 38 years. Patient 2 was a 58-year old male who died of maxillary sinus cancer and had worked in construction for 22 years. A pure-tone audiogram showed high-tone sensorineural hearing loss with c5-dip-type hearing disorder in both ears in Patient 1, and a high-tone abrupt form of sensorineural hearing loss in Patient 2. Pathological examination of the temporal bone revealed degeneration and disappearance of the organ of Corti at the basal turn and disappearance of cochlear neurons in both patients. Audio-cytocochleograms revealed hearing disorder consistent with the changes in the inner ear in both patients. Marked degeneration and disappearance of the organ of Corti and stria vascularis were present in patient 1. It is generally known that disorders of the organ of Corti for a long period is involved in the etiology of noise-induced hearing loss. This degeneration of the organ of Corti is produced at a basilar membrane with the maximum amplitude related to exposure to noise according to a physical and mechanical factors. Moreover, animal experiments have shown that exposure to noise decrease cochlear blood flow. In Patient 1 both the organ of Corti and the stria vascularis exhibited degeneration, suggesting that not only physical and mechanical factors but a cochlear circulatory disorder related to exposure to noise was involved in the etiology of the pathological changes in the temporal bone related to noise-induced hearing loss.

Chronic Pachymeningitis Presenting as Asymmetric Sensorineural Hearing Loss

The objective of this study was to characterize the auditory dysfunction associated with chronic pachymeningitis (inflammation of the dura mater). We conducted a university-based retrospective review. Three patients were identified who were diagnosed with chronic pachymeningitis after being referred for asymmetric sensorineural hearing loss. All patients were found to have other neurologic symptoms and signs during careful neurotologic evaluation. Two varieties of chronic pachymeningitis exist: a hypertrophic mass lesion and a linear dural thickening. Although the hypertrophic variety could be easily detectable by noncontrast magnetic resonance imaging (MRI), the linear form is only visible with the use of gadolinium enhancement. Chronic pachymeningitis is a rare form of sensorineural hearing loss that could portend an underlying disease of greater concern. Extensive evaluation is needed to exclude identifiable causes of chronic pachymeningitis, including infectious, neoplastic, and autoimmune diseases. The clinician should be aware that the evaluation of a patient with asymmetric sensorineural hearing loss involves more than simply ruling out an acoustic neuroma. Fast-spin echo MRI techniques without the use of gadolinium contrast could miss a number of potentially treatable diseases such as chronic pachymeningitis. Patients with asymmetric sensorineural hearing loss should be carefully evaluated for other neurologic findings, and imaging with enhanced MRI is recommended.

DFNB40, a recessive form of sensorineural hearing loss, maps to chromosome 22q11.21-12.1.

We report on a novel localization for a recessive form of deafness (DFNB), by linkage analysis in an Iranian consanguineous family. Affected individuals suffer from prelingual profound sensorineural hearing loss. Genome-wide analysis led to the characterization of a new locus, DFNB40, which maps to an approximately 9 Mb interval between markers D22S427 and D22S1144 at chromosome 22q11.21-12.1. Maximum lod score of 3.09 was obtained with D22S1174. Since the Bronx waltzer (bv) mouse mutant, characterized by waltzing behavior, deafness, and degeneration of cochlear inner hair cells, has been mapped to the syntenic region on murine chromosome 5, we suggest that DFNB40 and bv may result from orthologous gene defects.

Presbyacusis and the auditory brainstem response.

Age-related hearing loss (ARHL or presbyacusis) is an increasingly common form of sensorineural hearing loss (SNHL) as a result of changing demographics, and the auditory brainstem response (ABR) is a common experimental and clinical tool in audiology and neurology. Some of the changes that occur in the aging auditory system may significantly influence the interpretation of the ABR in comparison to the ABRs of younger adults. The approach of this review will be to integrate physiological and histopathological data from human and animal studies to provide a better understanding of the array of age-related changes in the ABR and to determine how age-related changes in the auditory system may influence how the ABR should be interpreted in presbyacusis. Data will be described in terms of thresholds, latencies, and amplitudes, as well as more complex auditory functions such as masking and temporal processing. Included in the review of data will be an attempt to differentiate between age-related effects that may strictly be due to threshold elevation from those that may be due to the aging process.

DFNB31, a recessive form of sensorineural hearing loss, maps to chromosome 9q32-34.

We report the identification of a novel locus responsible for an autosomal recessive form of hearing loss (DFNB) segregating in a Palestinian consanguineous family from Jordan. The affected individuals suffer from profound prelingual sensorineural hearing impairment. A genetic linkage with polymorphic markers surrounding D9S1776 was detected, thereby identifying a novel deafness locus, DFNB31. This locus could be assigned to a 9q32-34 region of 15 cM between markers D9S289 and D9S1881. The whirler (wi) mouse mutant, characterised by deafness and circling behaviour, maps to the corresponding region on the murine chromosome 4, thus suggesting that DFNB31 and whirler may result from orthologous gene defects.

Recurrent ischemia in symptomatic carotid occlusion: prognostic value of hemodynamic factors.

To identify hemodynamic factors that predict recurrence of ipsilateral cerebral ischemic events in patients with symptomatic carotid artery occlusion (CAO). The authors studied 117 consecutive patients with CAO and corresponding recent (</=6 months) ischemic symptoms of the brain or eye that were transient or at most mildly disabling. They determined, using Cox proportional hazards analysis, the prognostic value for recurrence of ipsilateral cerebral ischemic events of 1) clinical features believed to indicate hemodynamic compromise, 2) collateral blood flow pattern, and 3) transcranial Doppler CO(2)-reactivity. None of the 24 patients with symptoms of retinal ischemia alone had a recurrent cerebral ischemic event. In the 93 patients with cerebral ischemic symptoms on entry, recurrence of these symptoms was independently predicted by 1) the nature of the initial symptoms being of purported hemodynamic origin (limb-shaking, precipitation of symptoms by rising, exercise or low blood pressure, retinal claudication) (hazard ratio [HR] 3.8, 95% CI 1.5 to 9.5), 2) continuing symptoms after the CAO had been documented, but before inclusion in the study (HR 5.9, 95% CI 2.2 to 16.1), and 3) the presence of collateral blood flow via leptomeningeal vessels (HR 4.1, 95% CI 1.3 to 13.1). CO(2)-reactivity did not predict recurrence of cerebral ischemic events. Having cerebral in contrast to retinal ischemia, clinical features suggestive of hemodynamic compromise, continuing symptoms after demonstration of the CAO, and presence of leptomeningeal collaterals may help to identify patients with symptomatic CAO at high risk of future cerebral ischemia.

Autoimmune Inner Ear Disease (AIED)

A portion of idiopathic rapidly progressive sensorineural hearing loss (SNHL) cases can be attributed to an immunological process in the cochlea that has been designated "autoimmune inner ear disease" (AIED). AIED is characterized by asymmetric bilateral SNHL that progresses over weeks or months, is often accompanied by vestibular symptoms, and responds positively to immunosuppressant medication. Historically, the inner ear was thought to be protected from autoimmune pathology; however, recent evidence suggests that the perisacular tissue surrounding the endolymphatic sac contains the necessary components for an immunological reaction. In addition, the inner ear is capable of producing an autoimmune response to sensitized cells that can enter the cochlea from the circulatory system through the spiral modiolar vein. Thus, AIED can be organ-specific, originating within the inner ear, or non-organ-specific, originating outside the inner ear. Although AIED is a recognized form of SNHL that may be stabilized or even reversed by appropriate medication, it remains the subject of controversy and debate. This paper discusses the pathogenesis of AIED, diagnostic strategy and treatment procedures for AIED, and the role of the audiologist in patient management.

An autosomal recessive nonsyndromic form of sensorineural hearing loss maps to 3p-DFNB6.

Autosomal recessive nonsyndromic hearing loss (ARNSHL) is the most common form of congenitally acquired inherited hearing impairment. Although numerous loci are believed to exist, only five have been identified. Using a pooled genomic DNA screening strategy, we have identified a sixth locus, DFNB6, on 3p in the interval bounded by D3S1619 and D3S1766.

Familial Sensorineural Hearing Loss: A Correlative Study of Audiologic, Radiographic, and Vestibular Findings

A multidisciplinary approach to the study of a family with autosomal dominant sensorineural hearing loss is presented. The affected family members underwent extensive clinical and laboratory evaluation. They were found to have various degrees of bilateral congenital inner ear anomalies as imaged by computed tomography. The degree of structural abnormalities in the inner ears correlated with the severity of hearing impairment. Vestibular testing revealed nonspecific abnormalities generally correlating with audiologic and radiographic findings. This report presents a unique form of sensorineural hearing loss not previously described and is the first in-depth clinical study of nonsyndromal "Mondini dysplasia" occurring in a family.