peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome

ORPHA: 397744

Overview

human disease

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome, sourced from HPO and Orphanet clinical annotations.

Decreased nerve conduction velocityHyporeflexiaHoarse voiceMyopathyEMG: myopathic abnormalitiesIncreased variability in muscle fiber diameterLower limb muscle weaknessMildly elevated creatine kinaseBilateral sensorineural hearing impairmentProgressive distal muscle weaknessPeripheral neuropathyStructural foot deformityFatty replacement of skeletal muscleMitochondrial swellingAreflexiaTremorProximal muscle weaknessSeizureArthritis

Classification & Codes

Orphanet Code

ORPHA:397744
peripheral neuropathy-myopathy-hoarseness-hearing loss syndrome
OrphanetORPHA:397744
Treatments0 drug(s)
Symptoms on record19 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO