Comprehensive Hearing Loss NGS Panel

  • Panel Description
  • Test Description
  • CPT Codes
  • Resources

Panel Description

Hearing loss

Hearing loss refers to the partial or complete loss of hearing occurring bilaterally or unilaterally. Hearing loss may be conductive, sensorineural, or mixed. Approximately 80% of prelingual hearing loss is genetic. Hearing loss can be non-syndromic (~70% of cases) or syndromic (~30% of cases). While the majority of hearing loss is autosomal recessive, the condition can follow any inheritance pattern.

Whether age-related or present at birth, genetics often play a role in hearing loss. This panel includes genes associated with non-syndromic hearing loss conditions and syndromic forms of hearing loss including Usher Syndrome, Alport Syndrome, Jervell and Lange-Nielsen syndrome, Waardenburg syndrome, and branchiootorenal syndrome. Results of testing may help with diagnosis and risk assessment.This panel may be appropriate for anyone with a personal or a family history of hearing loss. Individuals or family members who have exhibited features such as failing a hearing test, having delayed speech development, being hard of hearing, and/or requiring hearing aids at a younger age than expected can benefit from this test.Genetic testing can help explain the cause of a patient’s hearing loss and help providers prepare for the onset of additional symptoms. If identified early, there may be treatment options to delay the onset of or ameliorate syndromic features.

Genetic testing for comprehensive hearing loss can:
  • Establish or confirm the appropriate diagnosis
  • Identify risks for additional related symptoms
  • Result in more personalized treatment and symptom management
  • Inform family members about their own risk factors
  • Provide options for family planning

Test Description

Print
  • Sequencing
  • Del/Dup
  • Rush / STAT
  • Exclude VUS
  • MCC
  • Duo/Trio
2 - 3 weeks
Call for details
ABHD12, ACTB, ACTG1, ADCY1, ADGRV1, AIFM1, ALMS1, AMMECR1, ANKH, ASAH1, ATP6V1B1, BCS1L, BDP1, BSND, BTD, CABP2, CACNA1D, CATSPER2, CCDC50, CD164, CDC14A, CDC42, CDH23, CEACAM16, CEP78, CHD7, CHSY1, CIB2, CISD2, CLDN14, CLIC5, CLPP, CLRN1, COCH, COL11A1, COL11A2, COL2A1, COL4A3, COL4A4, COL4A5, COL4A6, COL9A1, COL9A2, COL9A3, CRYL1, CRYM, DCDC2, DIABLO, DIAPH1, DIAPH3, DNMT1, DSPP, EDN3, EDNRB, ELMOD3, EPS8, ERCC2, ERCC3, ESPN, ESRRB, EYA1, EYA4, FGF3, FGFR1, FGFR2, FOXI1, GATA3, GIPC3, GJA1, GJB2, GJB3, GJB6, GPSM2, GRHL2, GRXCR1, GRXCR2, GSDME, HARS, HARS2, HGF, HOMER2, HOXB1, HSD17B4, ILDR1, KARS, KCNE1, KCNJ10, KCNQ1, KCNQ4, KITLG, LARS2, LHFPL5, LHX3, LOXHD1, LRP2, LRTOMT, MANBA, MARVELD2, MET, MIR96, MITF, MPZL2, MSRB3, MYH14, MYH9, MYO15A, MYO1C, MYO3A, MYO6, MYO7A, NARS2, NDP, NF2, NLRP3, OPA1, OSBPL2, OTOA, OTOF, OTOG, OTOGL, P2RX2, PAX3, PCDH15, PDZD7, PEX1, PEX6, PJVK, PMP22, PNPT1, POLR1C, POLR1D, POU3F4, POU4F3, PRPS1, PTPRQ, RDX, RIPOR2, ROR1, S1PR2, SEMA3E, SERPINB6, SIX1, SIX5, SLC17A8, SLC19A2, SLC26A4, SLC26A5, SLC4A11, SLC52A2, SLC52A3, SLITRK6, SMAD4, SMPX, SNAI2, SOX10, STRC, SYNE4, TBC1D24, TBL1X, TBX1, TCOF1, TECTA, TFAP2A, TIMM8A, TJP2, TMC1, TMEM132E, TMIE, TMPRSS3, TMPRSS5, TNC, TPRN, TRIOBP, TSPEAR, TWNK, TYR, USH1C, USH1G, USH2A, WFS1, WHRN ( 181 genes )
96% at 20x
Blood (two 4ml EDTA tubes, lavender top) or Extracted DNA (3ug in EB buffer) or Buccal Swab or Saliva (kits available upon request)
All sequencing technologies have limitations. This analysis is performed by Next Generation Sequencing (NGS) and is designed to examine coding regions and splicing junctions. Although next generation sequencing technologies and our bioinformatics analysis significantly reduce the contribution of pseudogene sequences or other highly-homologous sequences, these may still occasionally interfere with the technical ability of the assay to identify pathogenic variant alleles in both sequencing and deletion/duplication analyses. Sanger sequencing is used to confirm variants with low quality scores and to meet coverage standards. If ordered, deletion/duplication analysis can identify alterations of genomic regions which include one whole gene (buccal swab specimens and whole blood specimens) and are two or more contiguous exons in size (whole blood specimens only); single exon deletions or duplications may occasionally be identified, but are not routinely detected by this test. Identified putative deletions or duplications are confirmed by an orthogonal method (qPCR or MLPA). This assay will not detect certain types of genomic alterations which may cause disease such as, but not limited to, translocations or inversions, repeat expansions (eg. trinucleotides or hexanucleotides), alterations in most regulatory regions (promoter regions) or deep intronic regions (greater than 20bp from an exon). This assay is not designed or validated for the detection of somatic mosaicism or somatic mutations.

Gene Notes
OTOA Due to pseudogene interference, our current testing method is not able to reliably detect variants in exons 20-28 (NM_144672.3) in the OTOA gene.
STRC This test is capable of detecting the common STRC/CATSPER2 contiguous gene deletion. Due to pseudogene interference, other variants in STRC (e.g. partial gene deletions of STRC and sequence variants in STRC) are typically not evaluated.
TPRN Exon 1 of this gene is currently not amenable to current sequencing methods, therefore, sequencing and deletion/duplication analysis will not be performed for this region.
CPT Code 81252, 81324, 81325, 81404, 81405

NOTE:  The CPT codes listed on the website are in accordance with Current Procedural Terminology, a publication of the American Medical Association. CPT codes are provided here for the convenience of our clients. Clients who bill for services should make the final decision on which codes to use.

Resources

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Comprehensive Hearing Loss