Cystic Fibrosis and Hearing Loss
Literature Review: Introduction
The past two decades have presented technologies that help detect genetic hearing loss, especially among children, which include cystic fibrosis. Genetic variations lead to serious congenital hearing loss, which lowers both the patient and the family’s quality of life. This literature review aims to develop strategies for the detection and management of hearing loss for children with cystic fibrosis using existing genomic knowledge in children hearing loss. The implication of genetic technologies and the impact on management and diagnosis of the hearing loss will be developed in the review. The review will also evaluate how the technologies have impacted the general approach to the condition and the implementation of the discoveries in clinical practice to detect hearing loss, risks, and the essential follow-up and early intervention. The existing knowledge on the children hearing loss caused by cystic fibrosis will also be evaluated for insightful development of detection and management strategies. The resource for review is recent works of the last decade to enhance an up-to-date information background and practical intervention strategies suitable for the current situation of the hearing loss for children with cystic fibrosis.
Cystic Fibrosis Disease and Environment
Cystic fibrosis is a disease which is autosomal recessive in which the ion transportation in epithelial cells causes thick mucus. Thick and sticky mucus builds up around the lungs, kidney, pancreas, liver, and the digestive tract due to the disease (Tarshish et al. 2016). Hearing screening in children with cystic fibrosis is crucial in detecting the risk and the problem’s degree. Screening is crucial for children who have had exposure to aminoglycoside based on the increased prevalence of sensorineural hearing loss in the population (Farzal et al. 2016). The prescient environments include neonatal and prenatal infection exposures, while it may also be genetic. Children may carry a genetic variant that predisposes drug-induced ototoxicity of syndromic hearing loss, which may be manageable when detected early (Roland et al., 2016). However, variants associated with hearing loss may be hereditary genes in an autosomal to a recessive, mitochondrial pattern or autosomal to dominant. The gene that is affected or sometimes the specific variant correlates with distinct scientific phenotypes such as age onset, disease progression, affected auditory frequencies, and phenotypic severity (Roland et al., 2016). Each variant affects a specific part differently in the onset of childhood hearing loss.
Impact of Hearing Loss Caused by Cystic Fibrosis
Between 1 and 2 newborn babies per 1000 births suffer from hearing loss, which can essentially impact the child’s communication, education, and life quality. There is a high impact of hearing loss due to cystic fibrosis on communication and language acquisition among children and influences literacy skills and social well-being (Roland et al., 2016). The damage from ototoxic drugs is permanent, which results in psychological and social problems. Children’s language and learning development may be affected adversely even when the hearing loss intervention is restricted at high frequencies (Al Malky et al. 2011). The disease causes systemic issues such as insufficiency in the pancreas, sinopulmonary infections, and respiratory complications. Cystic fibrosis patients also suffer the more acute and chronic Pseudomonas aeruginosa bacterial infection, which makes the stated antibiotics necessary to use. Bilateral sensorineural hearing loss may sometimes be triggered by age-related hearing loss (ARHL), especially with high frequencies (Caumo et al. 2017). Congenital infections on mothers in the development of the embryo to the fetus may also result in sensorineural hearing loss (Tarshish et al. 2016). Such congenital infections include toxoplasmosis, syphilis, cytomegalovirus (CMV), rubella, and human immunodeficiency virus (HIV) (Liou et al. 2016).
Prognosis of the Condition
Cystic fibrosis has no cure and is unpreventable, although new treatment methods for the children improve quality of life and survival rate. Improved therapy has transformed cystic fibrosis from a high death risk in childhood to a chronic disease in which most patients live to adulthood. The World Health Organization records that 60% of hearing loss in children can be prevented early (WHO, 2016). The treatment of the recurrent infections resulting from cystic fibrosis requires patients to receive antibiotics proven to damage the ear’s sensory hair cells that may cause permanent hearing (Tarshish et al. 2016). Cystic fibrosis therapies may have ototoxic side effects on patients taking them routinely and increase sensorineural hearing loss risk. The commonly used antibiotics are intravenous aminoglycosides (IV AG) such as amikacin, tobramycin, and gentamicin due to their known ototoxic effect that destroys hearing and balance function (Al Malky et al. 2015).
Audiologists perform hearing tests on children with cystic fibrosis and examine related issues. The professionals also evaluate the presence, magnitude, and the cause of the hearing complications or loss on children and specialize at recognizing signs (Al Malky et al. 2015). Current research fails to address why some patients are at a higher risk of developing sensorineural hearing loss after receiving aminoglycosides (Schacht et al. 2012). Increased treatments also increase the risk of hearing loss of the patient. Audiologists and health experts suggest that early age screening could help detect the infection (Schacht et al. 2012). The ototoxic drugs are also approved for cystic fibrosis treatment due to the genetic mutation contributing to hearing loss for children with cystic fibrosis (Tarshish et al. 2016). Medicines such as treatments to neonatal infections and ototoxic medicines could also propagate hearing loss among children, therefore a factor in managing cystic fibrosis.
Strategies for Detection and Management of Hearing Loss
One of the most effective strategies in detecting and managing hearing loss in children is the improved perinatal medical care. The strategy has resulted in more surviving children, although some hearing complications may include loss of hearing. The high-risk factors identified with the hearing loss in children include extremely low birth weight, neonatal jaundice, and prematurity (Kriti et al. 2020). The factors that determine the child’s hearing loss’s impact include the age of onset, the magnitude of the hearing loss, the age of identification, and the living environment. Therefore, another effective strategy in managing cystic fibrosis in children is early detection (Farzal et al. 2016). Public awareness for ear care practices could also reduce ear infections, which lead to hearing loss and cystic fibrosis. Such awareness includes treating child ear complications at health facilities rather than home remedies and avoiding ear insertions that harm the inner ear parts. Management of the strategy is through community hearing and era care campaigns (World Health Organization, 2016).
Research Gaps Recommended for More Knowledge
High-frequency audiometry could solve the detection of hearing loss, especially resulting from ototoxic treatments. Early detection allows the known harmful medicines to be identified before the damage that affects speech frequencies. The relevant strategies identified for the detection and management of cystic fibrosis I children and prevention of hearing loss could be effective for survival (Kriti et al. 2020). After early age screening, immunization programs should be sustained to prevent possible ear infections such as congenital rubella, mumps, and meningitis. Such immunizations are most effective at the national level to ensure maximum population (World Health Organization, 2016). Supporting the maternity and child health initiative is also an effective management program that could prevent birth prematurity, hygienic practices, safe births, and proper neonatal infections. Healthcare workers and physicians also need prompt training on ear infections and the intervention through proper treatment options (Liou et al. 2016). Availing necessary technologies for treatments and managing cystic fibrosis, which may lead to hearing loss, is essential and promotes institutions that address the hearing loss ear complications. Raising awareness in communities on the stigma that hearing loss causes is also a management strategy for properly managing cystic fibrosis.
References
Al-Malky, G., Suri, R., Dawson, S., Sirimanna, T., Kemp, D. (2011). Aminoglycoside antibiotics cochleotoxicity in pediatric cystic fibrosis patients: a study using extended high-frequency audiometry and distortion product otoacoustic emissions. International Journal of Audiology; 50:112-22.
Al-Malky, G., Dawson, S.J., Sirimanna, T., Bagkeris, E. & Suri, R. (2015). High-frequency audiometry reveals a high prevalence of aminoglycoside ototoxicity in children with cystic fibrosis. Journal of Cystic Fibrosis, 14(2), 248-254.
Caumo, D.T., Geyer, L., Teixeira, R., Barreto, S. (2017). Hearing thresholds at high frequency in patients with cystic fibrosis: a systematic review, Brazilian Journal of Otorhinolaryngology, Volume 83, Issue 4.464-474.
Farzal, Z., Kou, Y.F., St. John, R., Shah, G.B., and Mitchell, R.B. (2016).The role of routine hearing screening in children with cystic fibrosis on aminoglycosides: A systematic review. The Laryngoscope, 126: 228-235.
Kriti, K., & Akanksha, N., & Wamik, A. (2020). Unveiling epidemiology and treatment strategies for efficient management of lung infection in cystic fibrosis. World Journal of Biology Pharmacy and Health Sciences.
Liou, T. G., Jensen, J. L., Allen, S. E., Brayshaw, S. J., Brown, M. A., Chatfield, B., Koenig, J., McDonald, C., Packer, K. A., Peet, K., Radford, P., Reineke, L. M., Otsuka, K., Wagener, J. S., Young, D., & Marshall, B. C. (2016). Improving performance in the detection and management of cystic fibrosis-related diabetes in the Mountain West Cystic Fibrosis Consortium. BMJ open diabetes research & care.
Roland, L., Fischer, C., Tran, K., Rachakonda, T., Kallogjeri, D., Lieu, J. E. (2016). Quality of life in children with hearing impairment: Systematic review and meta-analysis. Otolaryngology-Head and Neck Surgery 155(2): 208–219.
Schacht, J., Talaska, A. E., Rybak, L. P. (2012). Cisplatin and aminoglycoside antibiotics: Hearing loss and its prevention. The Anatomical Record (Hoboken) 295(11): 1837–1850.
Tarshish, Y., Huang, L., Jackson, F.I., Edwards, J., Fligor, B., Wilkins, A., Uluer, A., Sawicki, G., Kenna, M. (2016). Risk factors for hearing loss in patients with cystic fibrosis. Journal of the American Academy of Audiology, 27(1), 6-12.
World Health Organization (2016). Childhood Hearing Loss. Strategies for Prevention and Care. WHO Library Cataloguing-in-Publication Data