Annals of Indian Academy of Neurology
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ORIGINAL ARTICLE
Year : 2015  |  Volume : 18  |  Issue : 2  |  Page : 171-180

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


1 Department of Ear, Nose and Throat, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
2 Department of Otolaryngology, Head and Neck Surgery, Hamad Medical Corporation, Al Wakra Hospital, Doha, India
3 Department of Neurology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

Correspondence Address:
Anjali Lepcha
Department of Ear, Nose and Throat, Unit IV, Christian Medical College and Hospital, Vellore - 632 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.150578

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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.


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