CurrentCentenePolicy N/A

Genetic Testing: Hearing Loss (GJB2/GJB6 sequencing and multigene panels)

Defines medical necessity criteria for GJB2/GJB6 sequencing, deletion/duplication analysis, and multigene hearing-loss panels to establish a diagnosis of hereditary hearing loss for Centene-affiliated health plans.

Policy Summary

PayerCentene

PolicyGenetic Testing: Hearing Loss (GJB2/GJB6 sequencing and multigene panels)

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateNov 20, 2024

Key ActionObtain GJB2/GJB6 testing first for suspected autosomal recessive congenital hearing loss or obtain a multigene panel that includes these genes as an alternative.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

50%DFNB1 contribution

70-80%Nonsyndromic share

65%Prelingual genetic causes

Codes listedCPT examples

Coverage Criteria for Genetic Testing of Hereditary Hearing Loss

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Hereditary hearing loss testing criteria: A. The member/enrollee has hearing loss; AND B. There is no known acquired cause of the hearing loss (e.g., TORCH infections, bacterial infection, age-related or noise-related hearing loss).

Applies to GJB2 and GJB6 sequencing and/or deletion/duplication analysis or multigene panel analysis (CPT examples: 81252, 81479, 81430, 81431, 81254).

Diagnosis of hereditary hearing loss when member has hearing loss and no known acquired cause

Covered when ALL of the following are met

Diagnosis of hereditary hearing loss: The member has hearing loss AND there is no known acquired cause of the hearing loss (for example, TORCH infections, bacterial infection, age-related or noise-related hearing loss).

Applies to GJB2 and GJB6 sequencing and/or deletion/duplication analysis and multigene panel analysis (see CPT examples: 81252, 81479, 81430, 81431, 81254).

Investigational / Not covered indications

Investigational indication: GJB2 and GJB6 sequencing and/or deletion/duplication analysis or multigene panel analysis to establish a diagnosis of hereditary hearing loss is considered investigational for all other indications.

Investigational = not supported by this policy.

Testing for GJB2 and GJB6 sequencing and/or deletion/duplication analysis or multigene hearing-loss panels is considered investigational when it is not being used to establish a diagnosis of hereditary hearing loss that meets the policy criteria. In other words, any use outside the diagnostic indication described in this policy is investigational and not supported.

Coding References

Example CPT codes referencedCPT

81252	GJB2 gene sequencing and/or deletion/duplication analysis (example)
81479	Unlisted molecular pathology procedure / Multigene panel example referenced for deletion/duplication or sequencing
81430	Multigene panel analysis (example)
81431	Multigene panel analysis (example)
81254	GJB6 common variant analysis (example)

Example ICD-10 codes referencedICD-10

H90-H90.8	Conductive and sensorineural hearing loss diagnosis range (example ICD codes referenced)
H90.A-H90.A3	Other specified hearing loss (example ICD codes referenced)
H91.3-H91.93	Other and unspecified hearing loss (example ICD codes referenced)

Provider Actions, Prior Authorization, and Billing Notes

Prior Authorization

Prior authorization may be required for listed CPT codes

GJB2/GJB6 sequencing and multigene panel CPT examples cited in the policy include 81252, 81479, 81430, 81431, and 81254; per Health Plan procedures these test categories may require prior authorization before testing is performed or billed.

CPT examples: 81252, 81479, 81430, 81431, 81254
Prior authorization may be required per Health Plan procedures

Step Therapy

Stepwise testing approach

The policy endorses a stepwise evaluation for suspected hereditary hearing loss: either begin with GJB2 and GJB6 testing and, if negative, proceed to a multigene hearing-loss panel; or alternatively obtain a multigene panel that includes GJB2/GJB6 as the initial test—these strategies are considered reasonably equivalent.

Option 1: GJB2/GJB6 testing first, then multigene panel if negative
Option 2: Start with a multigene panel that includes GJB2/GJB6

Documentation Required

Coding and test documentation

When ordering and billing genetic testing, include the test name and link each CPT code to the appropriate ICD-10 diagnosis; providers should consult the most up-to-date professional coding guidance and the Concert Platform for comprehensive test listings prior to claims submission.

Include test name on the claim
Link CPT code(s) to an appropriate ICD-10 diagnosis (examples referenced: H90-H90.8, H90.A-H90.A3, H91.3-H91.93)
Reference current coding guidance and the Concert Platform for registered tests

Denial Risk

Investigational indications trigger denial

Testing for GJB2/GJB6 sequencing, deletion/duplication analysis, or multigene panel analysis for indications other than establishing a diagnosis of hereditary hearing loss is considered investigational and will not be supported under this policy.

Investigational = not supported by this policy; claims for other indications are subject to denial

Background

Hereditary hearing loss may be syndromic (hearing loss with other medical or physical findings) or nonsyndromic (hearing loss without other physical signs). Nonsyndromic forms account for approximately 70–80% of genetically determined deafness and are genetically heterogeneous, making it difficult in many cases to distinguish hereditary from acquired causes. DFNB1 (GJB2/GJB6) variants account for a large proportion of autosomal recessive nonsyndromic hearing loss, but many other genes can be implicated.

When a specific etiology is not suspected, the policy supports a stepwise evaluation: either begin with GJB2/GJB6 testing and proceed to a multigene panel if negative, or obtain a multigene panel that includes GJB2 and GJB6 as the initial test, since these strategies are considered reasonably equivalent.

Definitions

Nonsyndromic hearing loss — definition and statistic

DefinitionHearing loss not associated with other physical signs or symptoms.

Proportion of genetically determined deafnessAccounts for 70–80% of genetically determined deafness.

Clinical noteNonsyndromic cases are genetically heterogeneous; DFNB1 (GJB2/GJB6) explains a substantial portion of autosomal recessive cases but many other genes are implicated.

Syndromic hearing loss — definition

DefinitionHearing loss associated with other medical or physical findings, including visible external ear abnormalities.

RecognitionSyndromic hearing loss often is more readily recognized as hereditary because it occurs with multiple clinical manifestations.

Diagnostic implicationAssociated clinical findings can allow diagnosis of a syndrome, but findings may be absent at presentation and some loci can cause both syndromic and nonsyndromic loss.