Turner syndrome, also known as congenital ovarian hypoplasia, is a common genetic disorder due to an X chromosome aberration. Patients are all female, and due to their congenital ovarian hypoplasia, low ovarian function and insufficient estrogen secretion, they may exhibit pubertal secondary sexual characteristics failure, primary amenorrhea, short stature and somatoform deformities during development. Most patients first choose to consult an endocrinologist. However, about 60-90% of patients have a combination of otorhinolaryngologic disorders, which are often overlooked. 1. What is the relationship between Turner syndrome and otorhinolaryngological diseases? Due to the chromosomal defects in Turner syndrome, patients have various developmental disorders during the embryonic period. The main manifestations of otolaryngology include recurrent otitis media, hearing loss, vestibular dysfunction, inner ear malformations, auricular malformations, and cephalic and facial malformations such as high palatal vault, small jaw, and cervical webbing. Among them, the most obvious ones are auricular and cephalic developmental malformations, and the most neglected and serious symptom is deafness. 2. What can be the manifestations of deafness in combination with Turner syndrome? First, in general, these hearing losses can be divided into three types: conductive, sensorineural and mixed hearing loss. In children, repeated episodes of otitis media due to underdeveloped skull base structures can cause conductive hearing loss, which can affect the child’s ability to listen in class and cause some interpersonal difficulties. Although most children with conductive hearing loss will improve with age and development, the impact of poor hearing on speech development and learning may be irreparable. If allowed to develop, it may cause further aggravation of the hearing loss and eventual formation of adhesive otitis media – the serious sequelae of secretory otitis media. For adults, hearing loss mostly manifests itself as sensorineural hearing loss, which occurs silently and may have an impact on your life by the time you become aware of its existence. The causes of neurological hearing loss are unclear, but what is certain is that its incidence in Turner patients should not be underestimated. Some scholars statistics, at the age of 40, only about 40% of patients have the hearing level of normal 40-year-old women. 3. How should we detect these diseases in time? As mentioned earlier, if deafness symptoms are allowed to develop, then there will be irreversible consequences. What should we do? As early as 2007, the “Guidelines of the Turner Syndrome Study Group” already stated that hearing screening should be a screening program for children of all ages. Therefore, we recommend routine screening for otolaryngologic disorders to achieve early diagnosis and intervention. In order to achieve the diagnosis of these otolaryngological disorders, we need to perform audiological, inner ear function, and imaging examinations to clarify whether there is a combination of related symptoms, including: pure tone audiometry, acoustic conduction resistance, vestibular function examination, aberrant otoacoustic emission examination (DPOAE), CT examination of the temporal bone, and electronic laryngoscopy, etc. MRI may be required for special cases. 4. If these diseases are found, what treatments are required? In general, due to the specific nature of Turner syndrome, all patients need long-term follow-up regardless of whether they are diagnosed with a combination of ear, nose and throat disorders. It is recommended to have a hearing examination every six months to a year to observe the changes in hearing. For patients with definite otitis media, depending on the condition, different treatments are needed, such as tympanic membrane puncture and drainage, tympanic membrane placement or even middle ear mastoid surgery. Even cochlear implants can be performed. Therefore, we cannot ignore the combination of otorhinolaryngological diseases in Turner syndrome, and the earlier the diagnosis and intervention, the better it is to slow down the development of these comorbidities and minimize the adverse consequences.