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Abstract Introduction: Motor neuron disorders are a group of Neurodegenerative disorders which consist of degeneration of lower motor neurons and corticospinal tract. Sometimes there is involvement of medallary motor nuclei. In these group there is no involvement of sensory and autonomic systems. Madras pattern of Motor Neuron Disease (MMND) is mostly seen in southern India. Most of cases is being below 30 years of age. Classic features of disease include bulbar palsy with involvement of seventh to twelfth cranial nerves, sensori-neural deafness, weakness and atrophy of muscles of limbs and rarely optic atrophy and cerebellar signs. Case study: Our patient was a 16 years old girl who has sersori-neural hearing loss since 10 years before admission with a progressive course until 10 years of age. Weakness of limbs has started about 3 years before admission with a progressive course and bulbar palsy has started since 3 months before admission. Clinical and paraclinical studies were compatible with diagnosis of MMND. Results and Conclusion: MMND was reported rarely from other countries except India. Our case has characteristic features of MMND and there was no other report of MMND from Iran yet. Our patient had more progressive course rather than most of other reported cases.

Keywords: Amyotrophic Lateral Sclerosis, Hearing Loss, Sensorineural, Motor Neuron Disease

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