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Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

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Published on: January 25, 2016

Chronic acquired sensory neuron diseases.

A Damasceno¹, M C França, A Nucci

¹Department of Neurology, School of Medicine, State University of Campinas, Campinas, Brazil. alfredodamasceno@hotmail.com

European Journal of Neurology

|December 4, 2008

Summary

Sensory neuron diseases (SND) often present with sensory symptoms and ataxia. Early diagnosis relies on clinical and electrophysiological findings, as MRI may be normal initially.

Area of Science:

Neurology
Neuroscience
Peripheral Nervous System Disorders

Background:

Sensory neuron diseases (SND) are an increasingly recognized subgroup of peripheral nervous system disorders.
Understanding the diverse clinical, neurophysiological, and imaging features of SND is crucial for timely diagnosis and management.

Purpose of the Study:

To analyze the clinical, neurophysiological, and MRI characteristics of patients diagnosed with Sensory Neuron Diseases (SND).
To identify key diagnostic indicators and potential associations in SND patients.

Main Methods:

Retrospective review of clinical and electrophysiological data from 20 individuals meeting SND criteria.
Inclusion of neurological evaluation and cervical spine Magnetic Resonance Imaging (MRI) in the assessment.

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Analysis of cerebrospinal fluid (CSF) findings and correlation with disease duration and symptoms.

Main Results:

Sensory neuron diseases were associated with various conditions including dysimmune disorders, hepatitis C, B12 deficiency, and organophosphate intoxication; many cases were idiopathic.
Nineteen patients presented with sensory symptoms; worse ataxia correlated with longer disease duration.
Early electrophysiological assessments revealed widespread impairment in sensory nerve action potential amplitudes, while MRI showed posterior column hyperintensities in later stages (>3 years post-onset).

Conclusions:

Sensory neuron diseases typically manifest with sensory symptoms and ataxia, necessitating a high index of suspicion.
Inflammatory changes may be more pronounced early in the disease course, potentially benefiting from prompt immunotherapy.
Early diagnosis should prioritize clinical and electrophysiological data, as MRI findings may not be apparent in the initial stages.