All of the following are the classical presentation of Craniovertebral junction anomalies except -
Craniovertebral anomalies

Craniovertebral anomalies include congenital anomalies at the base of the skull and atlanto occipital region. It includes the occipito atlanto axial complex
Symptoms and signs of craniovertebral junction anomalies
Signs and symptoms of craniovertebral junction anomalies reflect the abnormalities and compromise of the following structures.


Pons
Medulla
Cervicomedullary junction and upper cervical spinal cord
Lower cranial nerves
Cervical nerve roots

Vascular supply to the neural structures

In craniovertebral anomalies, there is involvement of so many neurological structures that it presents with the confusing neurological picture.
A patient with congenital anomaly of craniovertebral junction usually presents with an abnormal physical appearance.
Congenital anomalies included in craniovertebral anomalies also include Klippel-Feil syndrome and Down's syndrome.
Findings in Klippel — Feil syndrome is 
Head cocked to one side 
Short neck
Abnormally low hairline posteriorly
Limitation of neck movement

Some more important facts about craniovertebral junction anomaly 
The most common neurological symptom is posterior occipital headache, that worsens with neck flexion and extension.
Myelopathy with involvement of pyramidal tract is also a very common symptom associated with cranio vertebral anomaly.
Most common cranial nerve dysfunction is hearing loss
Pupillary asymmetry usually results due to an abnormality of 2nd or 3rd nerve.
The 2nd nerve is cortical and 3rd nerve is related to the midbrain so quite obviously they will not get involved in craniovertebral junctional anomaly.
Pyramidal signs
Low hairline
Short neck
Pupillary asymmetry