Genetic Testing for Hereditary Hearing Loss (GJB2/GJB6 and Multigene Panels)
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Criteria for when genetic testing (GJB2 and GJB6 sequencing and/or deletion/duplication analysis or multigene hearing-loss panels) is considered medically necessary for members of Arizona Complete Health; includes coding examples, background, and related policy cross-references.
No material clinical or coverage changes in this revision.
Coverage Criteria for Genetic Testing in Hereditary Hearing Loss
GJB2/GJB6 sequencing and/or deletion/duplication analysis or multigene panel analysis (medically necessary indications)
Covered when ALL of the following are met
GJB2 and GJB6 sequencing and/or deletion/duplication analysis or multigene panel analysis is considered medically necessary under these conditions.
Unsupported indications
Not covered / Not supported when
Policy states evidence does not support testing for other indications.
Testing to establish hereditary hearing loss is limited by this policy to situations where the member has documented hearing loss and there is no known acquired cause (for example, TORCH infections, bacterial infection, or age- or noise-related hearing loss). Tests performed to diagnose hereditary hearing loss for indications outside these parameters are not supported by current evidence and may be denied. Providers should ensure clinical evaluation has excluded acquired causes before submitting genetic testing for authorization and coverage.
The policy specifies that GJB2 and/or GJB6 sequencing and deletion/duplication analysis, or multigene hearing-loss panels, are considered not supported by current evidence when used for indications other than a patient with documented hearing loss without an identified acquired cause. In other words, if the member does not meet both criteria—(1) presence of hearing loss and (2) absence of a known acquired cause—these genetic tests are not considered medically necessary under this policy.
Coding Examples and Diagnosis Codes
| 81252 | GJB2/GJB6 sequencing (example listed in policy) |
| 81254 | GJB6 sequencing (example listed in policy) |
| 81430 | CPT code example for multigene panel (policy lists as example) |
| 81431 | CPT code example for multigene panel (policy lists as example) |
| 81479 | Unlisted molecular pathology procedure (listed as common billing code example) |
| H90 | Hearing loss diagnoses range (policy references H90-H90.8) |
| H90.A | H90.A-H90.A3 (policy references) |
| H91.3 | H91.3-H91.93 (policy references) |
Provider Requirements, Prior Authorization, and Denial Risks
Prior authorization may be required (example CPT codes)
Prior authorization may be required for genetic testing codes commonly billed for hereditary hearing loss; examples listed in the policy include CPT codes 81252, 81254, 81430, 81431, and 81479. Inclusion of these codes is informational and does not guarantee coverage.
Step therapy: not applicable
No step therapy requirements are specified in this policy for GJB2/GJB6 sequencing, deletion/duplication analysis, or multigene panel testing for hereditary hearing loss.
Required clinical documentation and counseling
Document the presence of hearing loss and that acquired causes have been evaluated and excluded before genetic testing. Pre- and post-test genetic counseling is strongly advised and documentation that counseling occurred and a plan for returning results is recommended.
- Record objective evidence of hearing loss (audiology evaluation or equivalent).
- Document evaluation/exclusion of acquired causes (e.g., TORCH infections, bacterial infection, age-related or noise-related hearing loss).
- Document that pre-test counseling was provided, that the possibility of secondary/incidental findings was discussed, and the plan for returning results.
Denial risk when criteria are not met
Testing is limited to members who have hearing loss with no known acquired cause; requests that do not meet these criteria or lack documentation risk denial.
- Requests to establish hereditary hearing loss for indications other than hearing loss without an acquired cause are not supported by current evidence and may be denied.
- Absence of documentation demonstrating hearing loss or exclusion of acquired causes increases the likelihood of denial.
Background and Epidemiology
Approximately 65% of prelingual hearing loss in developed countries is attributable to genetic causes, and the DFNB1 locus (which includes the GJB2 and GJB6 genes) represents a substantial portion of nonsyndromic autosomal recessive hearing loss—estimated at about 50% of that group. Differentiation of genetic from acquired etiologies (for example, TORCH infections, cytomegalovirus, bacterial infection, or age- or noise-related hearing loss) is essential prior to ordering genetic testing; when history and exam do not point to a specific genetic cause, professional guidance supports initiating comprehensive hearing-loss gene panel testing.
Definitions and Counseling Expectations
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