hypogonadotropic hypogonadism-severe microcephaly-sensorineural hearing loss-dysmorphism syndrome

ORPHA: 293967

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 hypogonadotropic hypogonadism-severe microcephaly-sensorineural hearing loss-dysmorphism syndrome, sourced from HPO and Orphanet clinical annotations.

CryptorchidismHypogonadotropic hypogonadismMicropenisBifid uvulaMicrocephalyHypertelorismMicrognathiaProtruding earConvex nasal ridgeMyopiaGlobal developmental delayGynecomastiaPrimary amenorrheaDelayed pubertyInsulin-resistant diabetes mellitusSprengel anomalyHirsutismVisual field defectSeizureMotor delayHypertoniaSpecific learning disabilityShort statureOligohydramniosPes cavusOverlapping toeMicrocytic anemiaLower limb spasticityHighly arched eyebrowDelayed skeletal maturationGenu valgumBroad halluxCongenital sensorineural hearing impairmentContracture of the proximal interphalangeal joint of the 5th fingerMarked delay in bone ageHypoplasia of the tooth germCerebral dysmyelinationEarly-onset non-progressive night blindnessDecreased testicular sizeSevere postnatal growth retardationUnderdeveloped superior crus of antihelixBroad thumbModerate global developmental delayModerate intrauterine growth retardationFeeding difficultiesAbnormal optic disc morphologyDecreased corneal thickness

Classification & Codes

Orphanet Code

ORPHA:293967
hypogonadotropic hypogonadism-severe microcephaly-sensorineural hearing loss-dysmorphism syndrome
OrphanetORPHA:293967
Treatments0 drug(s)
Symptoms on record47 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO