Male infertility with azoospermia or oligozoospermia

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Male infertility with azoospermia or oligozoospermia due to single gene mutation

ORPHA:399805Disease

What it is

A rare, genetic male infertility due to a sperm disorder characterized by the absence of a measurable amount of spermatozoa in the ejaculate (azoospermia), or a number of sperm in the ejaculate inferior to 15 million/mL (oligozoospermia), resulting from a mutation in a single gene known to cause azoo- or oligo-spermia. Sperm morphology may be normal.

Key facts

Age of onset
Adolescent, Adult
Inheritance
Autosomal dominant, Autosomal recessive, X-linked recessive
Classified as
Disease

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

C14ORF39Disease-causing germline mutation(s)
CATIPDisease-causing germline mutation(s)
CCDC34Disease-causing germline mutation(s)
CFTRMajor susceptibility factor
CT55Disease-causing germline mutation(s)
DNAH10Disease-causing germline mutation(s)
DNHD1Disease-causing germline mutation(s)
FANCMDisease-causing germline mutation(s) (loss of function)
FBXO43Disease-causing germline mutation(s)
GCNADisease-causing germline mutation(s)
KLHL10Disease-causing germline mutation(s)
MEIOBDisease-causing germline mutation(s)
MOV10L1Disease-causing germline mutation(s)
MSH5Disease-causing germline mutation(s)
NANOS1Disease-causing germline mutation(s)
NR5A1Disease-causing germline mutation(s)
PDHA2Disease-causing germline mutation(s)
PNLDC1Disease-causing germline mutation(s)
RBBP7Disease-causing germline mutation(s)
RNF212Disease-causing germline mutation(s)
RPL10LDisease-causing germline mutation(s) (loss of function)
SHOC1Disease-causing germline mutation(s)
SOHLH1Major susceptibility factor
SPAG17Disease-causing germline mutation(s)
SPINK2Disease-causing germline mutation(s)
STAG3Disease-causing germline mutation(s)
SYCE1Disease-causing germline mutation(s)
TAF4BDisease-causing germline mutation(s)
TDRD9Disease-causing germline mutation(s)
TERB1Disease-causing germline mutation(s)
TERB2Disease-causing germline mutation(s)
TEX11Disease-causing germline mutation(s) (loss of function)
TEX14Disease-causing germline mutation(s)
TEX15Disease-causing germline mutation(s)
XRCC2Disease-causing germline mutation(s)
ZMYND15Disease-causing germline mutation(s)
ZSWIM7Disease-causing germline mutation(s)
SYCP3Candidate gene tested

ICD-10 codes

N46filed under a broader ICD-10 category — shared with 6 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 8530MONDO 0018393OMIM 108420OMIM 258150OMIM 270960OMIM 301077OMIM 301106OMIM 301137OMIM 305700OMIM 309120OMIM 613957OMIM 615081OMIM 615413OMIM 615841OMIM 615842OMIM 616950OMIM 617706OMIM 617707OMIM 617960OMIM 618086OMIM 618091OMIM 618110OMIM 618115OMIM 619108OMIM 619202OMIM 619379OMIM 619380OMIM 619515OMIM 619528OMIM 619585OMIM 619645OMIM 619646OMIM 619672OMIM 619673OMIM 619689OMIM 619696OMIM 619712OMIM 619799OMIM 619803OMIM 619805OMIM 619826OMIM 619828OMIM 619831OMIM 619867OMIM 619878OMIM 619937OMIM 619949OMIM 620084OMIM 620103OMIM 620170OMIM 620196OMIM 620222UMLS C5681165

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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