Johns Hopkins researchers are studying the frequency of otolaryngology-related surgery, such as the placement of tympanostomy tubes, in patients with achondroplasia.
Physicians in the Johns Hopkins Division of Pediatric Otolaryngology are advancing basic and clinical research and practice. The following are just a few examples.
Otolaryngology-related Surgery for Patients with Achondroplasia
“Patients with achondroplasia have a remarkably high incidence of otolaryngologic disease that requires surgical treatment, and our findings help us better understand this population.” – David Tunkel
Achondroplasia is the most common short-stature skeletal dysplasia, and ear, nose and throat disease is ubiquitous in patients with this form of dwarfism. Johns Hopkins otolaryngologist David Tunkel and geneticist Julie Hoover-Fong, and their colleagues on the Achondroplasia Natural History Study (CLARITY) team, studied more than 1,300 patients with achondroplasia who were treated at Johns Hopkins and three other U.S. centers during a 60-year period to measure the frequency of common otolaryngologic surgery.
Almost half of the patients had removal of tonsils, adenoids or both, and 11% of patients had this type of pharyngeal surgery more than once. About 57% of these patients had tympanostomy tubes placed, and almost one-third had tubes placed more than once. “Patients with achondroplasia have a remarkably high incidence of otolaryngologic disease that requires surgical treatment, and our findings help us better understand this population,” says Tunkel.
Multidisciplinary Care at Johns Hopkins Pediatric Thyroid Center
Children and adolescents with benign and malignant thyroid conditions have complex needs and require careful consideration and multidisciplinary care. The Johns Hopkins Pediatric Thyroid Center relies on a multidisciplinary team from Johns Hopkins Children’s Center and The Johns Hopkins Hospital to provide comprehensive care. From diagnosis through treatment, pediatric care is individualized and facilitated by Yasmin Akhtar from the Division of Pediatric Endocrinology and Diabetes, and Jonathan Walsh from the Division of Pediatric Otolaryngology.
“We understand that children require individualized care and special consideration,” says Walsh, “but our close affiliation with the expertise of the adult Johns Hopkins Thyroid and Parathyroid Center allows us a unique opportunity to offer children state-of-the-art multidisciplinary care and advanced surgical techniques, such as transoral thyroid surgery and radio frequency ablation.”
Clinician Communication with Families Regarding Pediatric Surgery
Recently, Johns Hopkins pediatric otolaryngologist–head and neck surgeon Emily Boss and her colleagues examined the dialogue that occurs during patient consultations for children with snoring, including how surgical clinicians responded to emotional communication from parents. They found that surgeons often responded and allowed space for parents to elaborate concerns, but response types varied according to the parent’s race, suggesting that implicit attitudes may influence how surgeons communicate with patients.
“Surgical care is historically focused more on technical skills and less so on interpersonal communication,” Boss says. “However, patient-centered communication is fundamentally critical in fostering trust and rapport between patients and surgeons, and it encourages patient and family engagement in the decision-making process. This research informs interventions that will train surgeons in culturally competent, empathic communication with families.”
How COVID-19 Affected Infant Hearing Loss Screening
The federal Early Hearing Detection and Intervention programs aim to have all children screened by age 1 month and diagnosed by 3 months, and for hearing intervention services to be enacted by age 6 months. The COVID-19 pandemic limited access and resources for patients and physicians. Recently, Walsh, Johns Hopkins pediatric otolaryngologist–head and neck surgeon Carolyn Jenks, pediatric nurse practitioner Melinda DeSell and colleagues sought to determine the impact of COVID-19 in Maryland on early hearing loss screening and intervention. They estimated that up to half of infants born with hearing loss in Maryland in 2021 may be delayed in diagnosis, and the findings are likely to be mirrored across the country, Walsh says.
“Much of the impact of these delays may not be realized for several years,” he adds, “and special attention and resources to mitigate the impacts are important for physicians in all states.”
Classifying Upper Lip Ties
Diagnosis and management of upper lip tie among infants and children in the U.S. have increased dramatically, yet there is still significant confusion, misinformation and uncertainty for physicians and families regarding this condition. Much of this is due to poorly defined diagnostic criteria that limits quality outcomes studies. To resolve this issue, Walsh and his colleagues performed the largest study to date describing upper lip anatomy in infants. The researchers used this information to create a novel classification system to help physicians diagnose and manage upper lip ties among infants.
“With this novel classification system,” Walsh says, “it will give the opportunity for more important studies to be done to help us understand the role the upper lip frenulum plays in infant feeding.”
Updated Tympanostomy Guidelines
Tympanostomy tube placement is the most common surgical procedure performed for children, yet there are wide variations in indications for this surgery, as well as differences in management of children with tubes in place. David Tunkel was part of a team that produced a revised, updated version this year of a tympanostomy tube clinical practice guideline published in 2013 by the American Academy of Otolaryngology–Head and Neck Surgery.
The updated guideline emphasizes the utility of tympanostomy tubes to improve hearing loss from middle ear fluid, especially for “at-risk” children who have long-term middle ear disease or other cognitive, developmental or sensory issues. The guideline recognizes the favorable natural history of recurrent ear infections even without surgery. The new update also offers information on the role of adenoidectomy as an adjunct to tube placement, and it provides educational materials for clinicians, patients and caregivers. It notes the need to measure outcomes objectively and prospectively for children who have this surgery.
To refer a patient, call 443-997-6467 or email JHOtoAccess@jhmi.edu.