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Hearing Loss | Overview


Topic: Hearing loss in patients that have CHARGE Syndrome
Principal Investigator: Dr. Margaret Kenna
Summary: Charge syndrome is an autosomal dominant disorder characterized by features represented in its acronym: coloboma, heart defect, atretic choanae, retarded growth or development, genital hypoplasia, and ear anomalies. CHARGE syndrome not only is associated with anomalies of the outer ear but also with changes of the inner ear structures and with hearing loss. The aim of this study is to assess the type, degree and development of hearing loss in this patient population. Moreover we will evaluate whether there is a correlation between the anatomical anomalies or results of genetic testing, the degree of hearing loss, and the inner ear anomalies.

Topic: Enlarged Vestibular Aqueduct
Principal Investigator: Dr. Margaret Kenna
Summary: Enlarged vestibular aqueduct syndrome is the most commonly identified inner ear bony malformation in patients with sensorineural loss. It is a congenital malformation of the temporal bone that causes early onset hearing loss and vestibular disturbances. It is also associated with multiple gene mutations, the most prominent of which is a mutation in SLC26A4 which encodes a transmembrane protein involved in chloride, iodide and bicarbonate ion transport. Improvements in the imaging studies used to evaluate the temporal bone in patients presenting with hearing loss have increased the frequency of this diagnosis. This growing population of patients warrants further study, especially considering long-term outcomes are not well defined. This study seeks to describe pediatric patients with EVA, and will be the largest study of this disease to date. In particular, we will exam the correlations between the anatomic structure of the vestibular aqueduct, audiometric findings, demographic variables, gene mutations, and clinical characteristics to better explore time dependent changes in patient phenotype. Notably, this study may influence future treatment protocol for these patients.

Topic: Hearing loss in patients that have Cystic Fibrosis
Principal Investigator: Dr. Margaret Kenna
Summary: Children with CF are at risk for developing sensorineural hearing loss (SNHL) due to the ototoxic drugs that they receive as part of their treatment. Despite the fact that this risk is recognized, gaps remain in what is known about why this sensory impairment occurs, and what can be done to prevent it. A pilot study from our group suggests that up to 17% of CF patients eventually will develop hearing loss. In this study we are examining the Otolaryngology and Audiology records for all children followed in the CF clinic. We are also collecting information on the types, dosages, and routes of administration of potentially drugs the patients have received to help manage their CF symptoms to identify potential risk factors associated with the development of SNHL. We hope to use this information to maintain or improve the quality of CF care while decreasing the incidence of hearing loss.
Additionally, we are conducting a study to try to identify causes of hearing loss in patients with Cystic Fibrosis. Patients with cystic fibrosis (CF) are at increased risk for sensorineural hearing loss (SNHL) due, at least in part, to the ototoxic side effects of routine CF therapies. However, the prevalence of SNHL and additional factors contributing to the development of SNHL are unknown. We are assessing the Cystic Fibrosis patient population’s noise exposure through surveying their use of MP3 players both at home and in the hospital. We would then like to look at their medical records for specific details regarding what ototoxic medication they are on, and dosage of medication in order to see if there is a confluence of components that make this population at higher risk for developing this sensory impairment.

Topic: Audiologically Profiling Patients with a Conductive Hearing Loss Due to Inner Ear Anomalies
Principal Investigator: Dr. GuangWei Zhou
Summary: This research seeks to examine the audiological profiles of patients with a conductive hearing loss, also know as an air/bone gap (the case where a person has better hearing when sound is conducted via bone versus via air). Traditionally, conductive hearing loss, indicated by air-bone gap in the audiogram, is linked to the external and/or middle ear pathologies, such as tympanic membrane perforation, otitis media with effusion, otosclerosis, etc. These pathological conditions are usually considered for a surgical procedure with great success in improving patients' hearing, or closing the air-bone gap. However, there are some patients with inner ear structural anomalies, such as superior semicircular canal dehiscence and enlarged vestibular aqueduct, can exhibit air-bone gap in their audiogram that mimics conductive hearing loss of middle/external ear origin. This study intends to identify the differences in the outcomes of audiological evaluation between patients with middle ear pathology and patients with inner ear anomaly. Findings from this study would help clinician, especially audiologists, in the diagnosis of conductive hearing loss.

Topic: Cholesteatoma
Principal Investigator: Dr. Dennis Poe
Summary: We are seeking to understand the best way to prevent and treat cholesteatoma. Hearing preservation and control of disease are important goals to address in the management of patients with chronic ear disease. We are comparing long-term hearing outcomes, rates of disease eradication, and rates of residual or recurrent disease in patients who have undergone different surgical approaches for cholesteatoma.