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Abstract

Objective

To report a case of bilateral sensorineural hearing loss in a patient with sensory ataxic neuropathy, dysarthria and ophthalmoparesis (SANDO).

Method

Case report and review of literature on mitochondrial disorders to facilitate understanding of the type of hearing loss.

Case report

We present a 37 year old lady diagnosed with SANDO, who was seen in the ENT department with a history of progressive hearing loss for 6 months. Audiogram showed bilateral trough shaped sensorineural hearing loss, dipping at 1 KHz. Earlier, this patient had presented to the neurology department with classical neurological signs of progressive sensory ataxia. Diagnosis of SANDO was confirmed by muscle biopsy and mitochondrial studies.

Conclusion

This is the first case report on SANDO documenting associated hearing loss in ENT literature.

Further studies are required to explain the pattern of hearing loss in these patients

Keywords

SANDO neuropathy sensorineural hearing loss mitochondria mutations

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Sensori-neural hearing loss in Sensory ataxic neuropathy,dysarthria and ophthalmoparesis (SANDO): A Case Report