The Jahrsdoerfer score was determined based on (1) stapes being present, (2) oval window being open, (3) round window being present and open, (4) middle ear space being large and favorable, (5) malleus-incus complex being present, (6) mastoid being well-pneumatized, (7) incus and stapes being connected, (8) facial nerve position, and (9) appearance of external ear being normal. The Jahrsdoerfer score, Marx grade, and extent of inferior displacement of the mastoid tegmen were determined.

Recent studies have attempted to document the clinical factors associated with CAA. Patients with narrow IS joint angles (120°), and the extent of inferior displacement of the mastoid tegmen is related to the severity of CAA. Although this grading system is widely used, other factors have been considered, such as the size of the incudostapedial (IS) joint angle and the extent of inferior displacement of the mastoid tegmen. The use of temporal bone computed tomography (TBCT) analysis is generally based on the Jahrsdoerfer grading system. Patient selection is critical when deciding on the treatment modality for CAA. Therefore, atresiaplasty and active middle ear implants (aMEI) are current treatment options for hearing rehabilitation in patients with CAA. Most patients with CAA combined with microtia exhibit conductive hearing loss because of a stenotic EAC and ossicular anomalies. Vestibular dysplasia (particularly in the semicircular canal) is estimated to be the most common inner ear anomaly in CAA patients. The incidence of inner ear anomalies reported in patients with CAA varies between 3% and 22%, and differs with higher incidences occurring with the presence of comorbidities and bilateral malformations. The prevalence of CAA is estimated to be one per 3,300 to 10,000, with the unilateral type reported to be three to five times more common than the bilateral type. CAA and microtia, which are relatively common in craniofacial microsomia, arise from developmental disorders involving the first and second branchial arches. Microtia is a congenital malformation of the external ear resulting in an auricular deformity and is typically observed with CAA.

For children who will have both microtia repair and atresiaplasty, the timing of one surgery may affect planning for the other surgery.Congenital aural atresia (CAA) is defined as a congenital malformation of the external auditory canal (EAC) and is often associated with anomalous features of the external, middle, and inner ear structures. In some cases, aural atresia repair is recommended though a procedure referred to as atresiaplasty. Problems with the ear canal could affect hearing or contribute to other problems such as ear infections or growth of cysts called cholesteatomas. Microtia and atresia can occur together in the same child. Aural atresia refers to cases in which the ear canal is narrow or absent. Microtia is a term which describes cases in which the outer portion of the ear, known as the pinna, is atypically shaped. Some children are born with visible differences to the external ear structures. A team might include otolaryngologists, plastic surgeons, audiologists, child anesthesiologists, and radiologists. Multiple professionals may be involved with providing care to a child who needs ear surgery. Additionally, specialists performing pediatric ear surgeries may be involved with other surgeries involving the face and skull. Certain hearing devices have internal components that need to be implanted requiring a surgical procedure. Common examples include surgery to repair various components of the outer or middle ear and procedures to address problems with the ear drum. Pediatric ear surgery may be recommended for a diverse range of conditions. Aspirin Exacerbated Respiratory Disease.Facial Plastic & Reconstructive Surgery.Petrous Apex Lesions, Cholesterol Granuloma, Cyst, Cholesteatoma.Minimally Invasive Endoscopic Skull Base Surgery.