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PubMed This is a summary of 12 peer-reviewed journal articles Updated
Audiology

The Care Plan: Managing Hearing and Thyroid Health

At a Glance

Pendred syndrome requires a coordinated care plan focusing on both hearing and thyroid health. Treatment often includes hearing aids or cochlear implants for progressive hearing loss, and medications like levothyroxine or surgery to manage thyroid issues such as hypothyroidism or goiter.

Managing Pendred syndrome is a marathon, not a sprint. Because the condition affects both the ears and the thyroid, care involves a team of specialists. The goal of treatment is to ensure your child has full access to sound for language development and to keep their metabolism balanced.

Hearing Management: Accessing the World of Sound

Because hearing loss in Pendred syndrome can be progressive or fluctuate, the “standard of care” evolves as your child grows.

  • Hearing Aids: For children with mild-to-moderate hearing loss, high-powered digital hearing aids are the first line of defense. These are frequently adjusted by an audiologist to match your child’s current hearing levels [1].
  • Cochlear Implants (CI): If hearing loss becomes severe-to-profound and hearing aids are no longer providing enough clear speech information, cochlear implants are recommended [2][3].
    • Success Rates: You can feel encouraged—children with Pendred syndrome and its associated inner ear malformations (like EVA) generally have excellent outcomes with cochlear implants. They often perform nearly as well as children with typical ear anatomy [4][5].
    • Surgical Note: During the implant surgery, doctors sometimes encounter a brief rush of inner ear fluid (often called a “gusher”) due to the enlarged vestibular aqueduct. Experienced surgeons easily manage this by carefully packing the surgical opening; it is a routine part of surgery for this condition and does not affect the long-term success of the implant [6][7].

Thyroid Management: Monitoring and Balance

Not every child with Pendred syndrome will need thyroid treatment immediately, but long-term monitoring is essential.

  • Levothyroxine: If your child develops hypothyroidism (an underactive thyroid), the standard treatment is a daily pill called levothyroxine. This is a synthetic version of the hormone your body naturally produces, and it is very effective at restoring normal energy and growth [8].
  • Managing a Goiter: If a goiter (neck swelling) becomes large enough to cause discomfort or difficulty swallowing, doctors may discuss surgery (thyroidectomy) to remove part or all of the thyroid gland. This has been shown to significantly improve quality of life for those with symptomatic swelling [9].
  • Regular Check-ups: Most children will need at least annual blood tests and occasionally a thyroid ultrasound to catch any changes early [10].

The Future: Molecular Medicine

While not yet a part of standard clinical care, the landscape of treatment is changing. Scientists are currently researching gene therapy and antisense oligonucleotide (ASO) therapy in laboratory models [11]. These experimental treatments aim to “fix” the genetic mistake at its source, potentially restoring pendrin function in the ear and thyroid [12]. While these are not yet available for patients, they represent a hopeful future.

Common questions in this guide

When does a child with Pendred syndrome need a cochlear implant?
Cochlear implants are recommended when hearing loss becomes severe or profound and standard hearing aids no longer provide clear speech information. Children with Pendred syndrome typically have excellent outcomes with these implants and perform similarly to children with typical ear anatomy.
Will my child need thyroid medication for Pendred syndrome?
Not all children with Pendred syndrome need thyroid medication right away. If your child develops an underactive thyroid (hypothyroidism), doctors will prescribe levothyroxine to balance hormone levels and support normal energy and growth.
How is a goiter treated in Pendred syndrome?
A goiter is a swelling of the thyroid gland in the neck. If it becomes large enough to cause discomfort or difficulty swallowing, treatments may include thyroid medication or a surgical procedure called a thyroidectomy to remove part or all of the gland.
Can surgery or gene therapy cure Pendred syndrome?
While surgery cannot cure the underlying genetic cause, cochlear implant surgery is highly successful at restoring access to sound. Future experimental treatments like gene therapy are currently being researched to fix the genetic mistake, but they are not yet available for patients.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Is my child currently gaining enough benefit from their hearing aids, or should we begin a cochlear implant evaluation?
  2. 2.What specific thyroid hormone levels (like TSH and Free T4) are you monitoring, and what would trigger a need for medication?
  3. 3.If we start levothyroxine for a goiter, will it help shrink the swelling, or is it only to balance hormone levels?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (12)
  1. 1

    Age at Intervention for Permanent Hearing Loss and 5-Year Language Outcomes.

    Ching TYC, Dillon H, Button L, et al.

    Pediatrics 2017; (140(3)) doi:10.1542/peds.2016-4274.

    PMID: 28864712
  2. 2

    Cochlear implantation in patients with Pendred syndrome.

    Patterson TE, Gonzalez VB, Carron JD

    American journal of otolaryngology 2021; (42(6)):103087 doi:10.1016/j.amjoto.2021.103087.

    PMID: 34029917
  3. 3

    Patients with Pendred syndrome: is cochlear implantation beneficial?

    van Nierop JW, Huinck WJ, Pennings RJ, et al.

    Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery 2016; (41(4)):386-94 doi:10.1111/coa.12532.

    PMID: 26331303
  4. 4

    Audiological and Speech Outcomes of Cochlear Implantation in Inner Ear Malformation Compared to the Normal Inner Ear.

    Potdukhe K, Vishwakarma R, Rao S, Vishwakarma C

    Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India 2023; (75(3)):1870-1875 doi:10.1007/s12070-023-03760-y.

    PMID: 37636621
  5. 5

    Speech development in young children with Mondini dysplasia who had undergone cochlear implantation.

    Qi S, Kong Y, Xu T, et al.

    International journal of pediatric otorhinolaryngology 2019; (116()):118-124 doi:10.1016/j.ijporl.2018.10.013.

    PMID: 30554681
  6. 6

    [The value of HRCT in predicting cerebrospinal fluid gusher during cochlear implantation].

    Liu Z, Lin X, Huang H, et al.

    Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery 2024; (38(5)):421-425 doi:10.13201/j.issn.2096-7993.2024.05.014.

    PMID: 38686481
  7. 7

    Cochlear Implantation in Pediatric Patients With Enlarged Vestibular Aqueduct: A Systematic Review.

    Benchetrit L, Jabbour N, Appachi S, et al.

    The Laryngoscope 2022; (132(7)):1459-1472 doi:10.1002/lary.29742.

    PMID: 34233033
  8. 8

    Mutation screening of the SLC26A4 gene in a cohort of 192 Chinese patients with congenital hypothyroidism.

    Fu C, Zheng H, Zhang S, et al.

    Archives of endocrinology and metabolism 2016; (60(4)):323-7.

    PMID: 26886089
  9. 9

    The Impact of Post-Thyroidectomy Neck Stretching Exercises on Neck Discomfort, Pressure Symptoms, Voice and Quality of Life: A Randomized Controlled Trial.

    Thorsen RT, Døssing H, Bonnema SJ, et al.

    World journal of surgery 2022; (46(9)):2212-2222 doi:10.1007/s00268-022-06610-0.

    PMID: 35637354
  10. 10

    Case of delayed presentation of Pendred syndrome with a large goitre causing a life-threatening airway obstruction.

    Sasaki T, Onaga R, Koshu R

    BMJ case reports 2022; (15(6)) doi:10.1136/bcr-2022-250990.

    PMID: 35667695
  11. 11

    Postnatal Slc26a4 gene therapy improves hearing and structural integrity in a hereditary hearing loss model.

    Tsai YH, Wu PY, Chuang YC, et al.

    The Journal of clinical investigation 2026; (136(8)).

    PMID: 41701544
  12. 12

    Rescue of mis-splicing of a common SLC26A4 mutant associated with sensorineural hearing loss by antisense oligonucleotides.

    Feng P, Xu Z, Chen J, et al.

    Molecular therapy. Nucleic acids 2022; (28()):280-292 doi:10.1016/j.omtn.2022.03.015.

    PMID: 35433113

This page explains treatment options for Pendred syndrome for educational purposes. Always consult with your child's audiologist and endocrinologist for a personalized care plan.

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