Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India

• Main Authors: Anjali Lepcha, Reni K Chandran, Mathew Alexander, Ann Mary Agustine, K Thenmozhi, Achamma Balraj
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2015-01-01
• Series: Annals of Indian Academy of Neurology
• Subjects: Auditory neuropathy; Charcot- marie- tooth disease; consanguinity; Friedreich ataxia; genetic counseling; hereditary sensory; motor neuropathy; neurologic dysfunction; Refsum disease; sensorineural hearing loss
• Online Access: http://www.annalsofian.org/article.asp?issn=0972-2327;year=2015;volume=18;issue=2;spage=171;epage=180;aulast=Lepcha

Aims: To find out the prevalence and types of neurological abnormalities associated in auditory neuropathy spectrum disorder in a large tertiary referral center. Settings and Design: A prospective clinical study was conducted on all patients diagnosed with auditory neuropathy spectrum disorder in the ear, nose, and throat (ENT) and neurology departments during a 17-month period. Patients with neurological abnormalities on history and examination were further assessed by a neurologist to determine the type of disorder present. Results: The frequency of auditory neuropathy spectrum disorder was 1.12%. Sixty percent were found to have neurological involvement. This included cerebral palsy in children, peripheral neuropathy (PN), spinocerebellar ataxia, hereditary motor-sensory neuropathy, spastic paresis, and ponto-bulbar palsy. Neurological lesions did not present simultaneously with hearing loss in most patients. Sixty-six percent of patients with auditory neuropathy spectrum disorder were born of consanguineous marriages. Conclusions: There is a high prevalence of neurological lesions in auditory neuropathy spectrum disorder which has to be kept in mind while evaluating such patients. Follow-up and counselling regarding the appearance of neuropathies is therefore important in such patients. A hereditary etiology is indicated in a majority of cases of auditory neuropathy spectrum disorder.